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Objective: Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS., Study Design: Retrospective study., Setting: Tertiary care center., Methods: We included pediatric patients diagnosed with CAS on temporal bone CT and with available temporal bone MRI. For each patient, an otolaryngologist and a pediatric neuroradiologist measured the cochlear aperture width on CT to confirm CAS (cochlear aperture < 1.4 mm) and assessed the status of the cochlear nerve on MRI., Results: Fifty-five patients, representing 65 ears, had CAS on CT measurement. Median cochlear aperture width in CAS ears was 0.70 mm (interquartile range [IQR]: 0.40-1.05 mm) versus 2.00 mm in non-CAS ears (IQR: 1.80-2.30 mm, P < .001). CND was found in 98.5% (n = 64/65) of CAS ears, while a normal cochlear nerve was found in 1.5% (n = 1/65) of CAS ears., Conclusion: CND is highly prevalent among pediatric patients with CAS. This suggests that MRI may not be needed to assess for CND in USNHL patients with CAS, as initial CT may provide sufficient information to determine cochlear implant candidacy. We recommend thoughtful shared decision-making with parents of USNHL patients when determining whether to pursue MRI in the setting of a CAS diagnosis., (© 2024 The Authors. Otolaryngology–Head and Neck Surgery published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology–Head and Neck Surgery Foundation.)

Background: No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported., Aims/objectives: We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs)., Materials and Methods: Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed., Results: The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively., Conclusions and Significance: Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.

Background: Primary hereditary microcephaly (MCPH) comprises a large group of autosomal recessive disorders mainly affecting cortical development and resulting in a congenital impairment of brain growth. Despite the identification of >25 causal genes so far, it remains a challenge to distinguish between different MCPH forms at the clinical level., Methods: 7 patients with newly identified mutations in CDK5RAP2 (MCPH3) were investigated by performing prospective, extensive and systematic clinical, MRI, psychomotor, neurosensory and cognitive examinations under similar conditions., Results: All patients displayed neurosensory defects in addition to microcephaly. Small cochlea with incomplete partition type II was found in all cases and was associated with progressive deafness in 4 of them. Furthermore, the CDK5RAP2 protein was specifically identified in the developing cochlea from human fetal tissues. Microphthalmia was also present in all patients along with retinal pigmentation changes and lipofuscin deposits. Finally, hypothalamic anomalies consisting of interhypothalamic adhesions, a congenital midline defect usually associated with holoprosencephaly, was detected in 5 cases., Conclusion: This is the first report indicating that CDK5RAP2 not only governs brain size but also plays a role in ocular and cochlear development and is necessary for hypothalamic nuclear separation at the midline. Our data indicate that CDK5RAP2 should be considered as a potential gene associated with deafness and forme fruste of holoprosencephaly. These children should be given neurosensory follow-up to prevent additional comorbidities and allow them reaching their full educational potential., Trial Registration Number: NCT01565005., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives/hypothesis: To investigate the prevalence and course of cochlear obliteration according to microsurgical approach to inform clinical decision making regarding optimal timing of cochlear implantation., Study Design: Retrospective radiologic review and chart review., Methods: Patients who underwent microsurgical resection of vestibular schwannoma (VS) with a minimum of two available postoperative magnetic resonance imaging (MRI) scans were analyzed. The prevalence and timing of cochlear and labyrinthine obliteration was classified using relevant MRI sequences., Results: MRI studies in 60 patients were analyzed: 20 translabyrinthine (TL), 20 retrosigmoid, and 20 middle fossa (MF) cases. The first and last postoperative MRI was obtained a median of 3.4 months (interquartile range (IQR), 3.0-3.7 months) and 35 months (IQR, 27-83 months) after surgery, respectively. At the time of the first postoperative MRI, 21 (35%) patients had partial basal turn obliteration, and none of the patients had complete basal turn obliteration. At the time of the last postoperative MRI, six (10%) patients had partial basal turn obliteration and 17 (28%) patients had complete basal turn obliteration. The pattern of partial or complete basal turn obliteration differed significantly among all three surgical approaches (P < .001). Specifically, the risk of partial or complete obliteration of the basal turn was highest in the TL cohort and lowest in the MF cohort., Conclusions: The prevalence and timing of cochlear obliteration after VS microsurgery varies significantly according to surgical approach. The risk of early and complete obliteration is highest in the TL group and lowest in the MF cohort. These data may inform clinical decision making regarding optimal timing of cochlear implantation in patients with advanced hearing loss after microsurgical resection., Level of Evidence: 4 Laryngoscope, 130:474-481, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

A 67-year-old woman was referred to the otolaryngology service after presenting to the emergency department for dizziness and loss of balance. She reported several similar episodes over the past years. Physical examination was unremarkable. A temporal bone CT scan revealed dehiscence between the bony carotid canal and the cochlea resulting in the diagnosis of carotid-cochlear dehiscence (CCD). CCD is an extremely rare condition involving the thinning of the bony canal separating the internal carotid artery from the cochlea. CCD is best diagnosed with temporal bone CT scan. Treatment options include observation as well as chemical or surgical labyrenthectomy. Despite similar clinical and diagnostic characteristics of reported CCD cases, general trends and consensus on treatment options cannot be ascertained due to the extreme rarity of this condition. Regardless of these limitations, CCD is a critical diagnosis as it mimics other inner ear conditions and poses a potential, significant surgical risk for the otolaryngologist., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To examine when cochlear fibrosis occurs following a translabyrinthine approach for vestibular schwannoma resection, and to determine the safest time window for potential cochlear implantation in cases with a preserved cochlear nerve., Methods: This study retrospectively reviewed the post-operative magnetic resonance imaging scans of patients undergoing a translabyrinthine approach for vestibular schwannoma resection, assessing the fluid signal within the cochlea. Cochleae were graded based on the Isaacson et al. system (from grade 0 - no obstruction, to grade 4 - complete obliteration)., Results: Thirty-nine patients fulfilled the inclusion criteria. The cochleae showed no evidence of obliteration in: 75 per cent of patients at six months, 38.5 per cent at one year and 27 per cent beyond one year. Most changes happened between 6 and 12 months after vestibular schwannoma resection, with cases of an unobstructed cochlear decreasing dramatically, from 75 per cent to 38.5 per cent, within this time., Conclusion: The progress of cochlear obliteration that occurred between 6 and 12 months following vestibular schwannoma resection indicates that the first 6 months provides a safer time window for cochlear patency.

Background: In sudden and asymmetrical progressive sensorineural hearing loss (SNHL), magnetic resonance imaging (MRI) is required to evaluate retrocochlear pathology and, with recent advances in MRI techniques, inner ear pathology. Given the limited literature regarding inner ear pathology associated with SNHL, we aimed to assess the incidence of retrocochlear and inner ear pathology, and congenital malformation on MRI in sudden SNHL (SSNHL) and progressive SNHL., Methods: A total of 987 acoustic neuroma (AN) protocol MRI internal acoustic meatus studies performed at our institution to investigate SNHL between January 2013 and December 2015 inclusive were identified. Following categorization for indication of SSNHL versus progressive asymmetrical SNHL, MRIs with retrocochlear or inner ear abnormality, congenital malformation or other otology-related abnormality were identified, and further data were collected for these patients including patient demographics, associated symptomatology, management and outcomes., Results: In SSNHL, aetiological abnormality on MRI was identified for 6.9% patients with AN present on 4% overall. 3.2% of MRIs for progressive asymmetrical SNHL identified a causative lesion with 2.3% of scans overall diagnosing AN. The incidence of congenital inner ear malformation on MRI in the setting of SSNHL and progressive asymmetrical SNHL are 1.7% and 0.6%, respectively., Conclusion: This is the first retrospective study of inner ear MRI abnormalities in both SSNHL and progressive asymmetrical SNHL in Australia and one of the largest cohorts published in the literature to date. MRI must be performed in the setting of SNHL to ensure aforementioned and rarer causative lesions are identified., (© 2018 Royal Australasian College of Surgeons.)

Purpose: The timing of CI for postmeningitic deafness is controversial and differential outcomes have been reported. To review and share our surgical and auditory outcomes., Materials and Methods: 17 patients with ossified cochleas who received CI were enrolled. Clinical data including the cause of cochlear ossification, preoperative examination, onset of deafness, age at implantation, surgical findings, and relevant auditory outcomes was analysed., Results: Cochlear ossification was observed in 53% of patients with HRCT, whereas the corresponding value for MRI was 59%. Patients in both stage I and II received complete insertion of the electrode array, however, stage III patients only received partial insertion. 1 patient in stage II received bilateral CI. Hearing tests showed increased average hearing threshold for stage III patients than those in stage I and II (P < 0.05). CAP scores were much lower for stage III patients than those in stage I and II (P < 0.05). Postlingual deafness patients showed higher SIR scores than prelingual deafness children (P < 0.05)., Conclusions: HRCT and MRI have comparable value in predicting the occurrence of ossification in cochleas. We recommend fast surgical intervention in the patients with bilateral profound postmeningitic deafness. If possible, bilateral cochlear implantation is recommended., (Copyright © 2018 Elsevier Inc. All rights reserved.)

The most frequent sequelae following a translabyrinthine approach for vestibular schwannoma resection is complete hearing loss on the affected side. Such patients could benefit from a cochlear implant, provided that two essential requisites are met before surgery: a preserved cochlear nerve and a patent cochlea to accommodate the electrode array. The goal of our study is to determine the prevalence and extent of cochlear ossification following a translabyrinthine approach. Postoperative MRI of 41 patients were retrospectively reviewed. Patients were classified according to the degree of cochlear obliteration into three groups (patent cochlea, partially obliterated cochlea and totally obliterated cochlea). The interval between surgery and the first MRI was studied as well as its relationship with the rate of cochlear ossification. At first postoperative MRI (mean interval of 20 months), 78% of patients showed some degree of cochlear ossification. Differences were found in the time interval between surgery and first MRI for each group, showing a smaller interval of time the patent cochlea group (p > 0.05). When MRI was performed before the first year after surgery, a larger rate of patent cochlea was found (p > 0.05). The present study suggests that cochlear ossification is a time-depending process, whose grounds are still to be defined., (Copyright © 2018 Società Italiana di Otorinolaringologia e Chirurgia Cervico-Facciale, Rome, Italy.)

Objective: The aim of the current study is to evaluate audiologic and radiologic findings of cochlear hypoplasia which is a subgroup of inner ear malformations., Methods: This study was a prospective clinical study and based on voluntary participation from cases with cochlear hypoplasia diagnosis. The study was conducted at Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from an inner ear malformations database. Inclusion criteria were having cochlear hypoplasia for at least one ear. There were 66 subjects with an age range of 12 months and 60 years 5 months. For each subject, pure tone audiometry and tympanometry were applied according to chronological and cognitive age. And also, auditory brainstem response test was applied to when it is need. Subjects' radiologic results were reevaluated to confirm cochlear hypoplasia, cochlear nerve and cochlear aperture., Results: Cochlear hypoplasia types were statistically significantly different in terms of HL degree. This difference was caused by cochlear hypoplasia type IV group being was statistically different from the other three groups. Like with degree of HL, cochlear hypoplasia groups were statistically different from other three groups in terms of type of hearing loss. Cochlear aperture and cochlear nerve status showed variation according to cochlear hypoplasia type but these differences were not statistically approved., Conclusions: In the current study, incidence of cochlear hypoplasia was 23.5% in all inner ear malformation. With this study, it was seen that subtypes of cochlear hypoplasia showed variability in terms of degree and type of hearing loss and also cochlear aperture and cochlear nerve status. Especially cochlear hypoplasia type IV differs from other three cochlear hypoplasia types., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Purpose: The goal of this retrospective study was to investigate the differential diagnosis of endolymphatic hydrops in patients with Meniere's disease (MD) symptoms by using magnetic resonance imaging (MRI) with intravenous injection of gadolinium chelate and delayed acquisition., Material and Method: Two hundred patients (133 women, 67 men; mean age=67.2±11 ([SD] years) with unilateral MD underwent MRI at 3-T, between 4.5 and 5.5hours after intravenous administration of gadoterate meglumine at a dose of 0.1mmol/kg. MR images were analyzed for the presence of saccular hydrops, perilymphatic fistulae, inner ear malformations, semicircular canal (SCC) abnormal enhancement and brain lesions. We also tested the potential relationship between past history of gentamicin intratympanic administration and perilymphatic fistula presence and SCC aspect., Results: Saccular hydrops were found in 96/200 patients with MD (48%). Three patients (1.5%) had perilymphatic fistulas associated with saccular hydrops, as confirmed by surgery. There was a correlation between the presence of perilymphatic fistula and past history of intratympanic gentamicin administration (P=0.02). We detected inner ear malformations in 5 patients (2.5%), SCC local enhancement in 15 patients (7.5%) always on the same side than the clinical symptoms of MD. There was a correlation between the presence of SCC abnormal enhancement and past intratympanic gentamicin administration (P=0.001). Five patients (2.5%) had brain lesions along central cochleovestibular pathways., Conclusion: MRI may reveal brain lesions, SCC abnormalities and perilymphatic fistulae in patients with clinical MD., (Copyright © 2017 Editions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Importance: Pediatric-onset unilateral and asymmetric sensorineural hearing loss (SNHL) is a common condition, but in most patients, the cause remains unclear; thus, determination of the hearing outlook is difficult., Objective: To analyze the etiologic and audiologic characteristics of pediatric-onset unilateral and asymmetric SNHL., Design, Setting, and Participants: In this retrospective cohort study performed from January 1, 2008, through December 31, 2016, patients at a tertiary referral center who were diagnosed with pediatric-onset unilateral or asymmetric SNHL were divided into 3 groups according to their hearing levels: unilateral hearing loss with scaled-out levels (UHL-SO), unilateral hearing loss with residual hearing (UHL-RH), and asymmetric hearing loss (AHL)., Main Outcomes and Measures: Basic demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and compared among patients of the 3 groups., Results: A total of 133 patients (mean [SD] age, 9.1 [10.9] years; 63 [47.4%] male and 70 [52.6%] female), including 50 with UHL-SO, 42 with UHL-RH, and 41 with AHL, were enrolled for analyses. Of 50 patients with UHL-SO, 49 (98.0%) had stable hearing levels with time, whereas 10 of 42 patients with UHL-RH (23.8%) and 18 of 41 patients with AHL (43.9%) revealed progressive or fluctuating hearing loss. Inner ear malformations detected with temporal bone high-resolution computed tomography, particularly cochlear aperture stenosis, were detected at higher rates in patients with UHL-SO (9 of 31 [29.0%]) and UHL-RH (6 of 24 [25.0%]) than in those with AHL (1 of 30 [3.3%]). In contrast, screening for mutations in 3 common deafness genes-GJB2, SLC26A4, and MTRNR1-achieved definite diagnosis in a higher percentage of patients with AHL (10 of 37 [27.0%]) than patients with UHL-SO (0 of 33) and UHL-RH (1 of 25 [4.0%])., Conclusions and Relevance: The UHL-SO and UHL-RH conditions share a common or similar etiopathogenesis different from that of AHL. Imaging studies and genetic testing might be prioritized during the respective general etiologic workups for patients with UHL and AHL. Regular hearing checkups are warranted for patients with UHL and AHL because a certain proportion of patients might sustain progression in SNHL.

There is substantial variability in speech recognition ability across patients with cochlear implants (CIs), auditory brainstem implants (ABIs), and auditory midbrain implants (AMIs). To better understand how this variability is related to central processing differences, the current electroencephalography (EEG) study compared hearing abilities and auditory-cortex activation in patients with electrical stimulation at different sites of the auditory pathway. Three different groups of patients with auditory implants (Hannover Medical School; ABI: n = 6, CI: n = 6; AMI: n = 2) performed a speeded response task and a speech recognition test with auditory, visual, and audio-visual stimuli. Behavioral performance and cortical processing of auditory and audio-visual stimuli were compared between groups. ABI and AMI patients showed prolonged response times on auditory and audio-visual stimuli compared with NH listeners and CI patients. This was confirmed by prolonged N1 latencies and reduced N1 amplitudes in ABI and AMI patients. However, patients with central auditory implants showed a remarkable gain in performance when visual and auditory input was combined, in both speech and non-speech conditions, which was reflected by a strong visual modulation of auditory-cortex activation in these individuals. In sum, the results suggest that the behavioral improvement for audio-visual conditions in central auditory implant patients is based on enhanced audio-visual interactions in the auditory cortex. Their findings may provide important implications for the optimization of electrical stimulation and rehabilitation strategies in patients with central auditory prostheses. Hum Brain Mapp 38:2206-2225, 2017. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Background: The 6p25 deletion syndrome is one of the many syndromes with both hearing impairment as well as vision impairment. However, the audiometric characteristics and radiological findings of patients with 6p25 deletions are only scarcely described in literature. This study focused on characterizing the audiometric and radiological features of a Belgian family with a chromosome 6p25 deletion., Objective: To evaluate the hearing impairment, audiometric testing and radiological examination of the temporal bones in 3 family members with a 3.4 Mb deletion in chromosome band 6p25., Results: All 3 family members demonstrated slowly progressive sensorineural or mixed hearing impairment. Radiologic examination revealed thickened and sclerotic stapes in all patients and a minor internal partition type II of the cochlea in 2 patients., Conclusion: There is a significant phenotypic variability within and among families with the 6p25 deletion syndrome. A thorough genotype-phenotype correlation is difficult because of the small number of affected patients and the limited clinical data available. More clinical data of families with 6p25 deletions need to be published in order to create a reliable and precise phenotypic characterization. However, our findings can facilitate counseling of hearing impairment caused by 6p25 deletions., (© The Author(s) 2016.)

Objectives/hypothesis: Pathologic third window lesions, such as superior semicircular canal dehiscence syndrome (SCDS) or large vestibular aqueduct syndrome (LVAS), cause several auditory and vestibular symptoms, which might affect perilymphatic pressure and induce endolymphatic hydrops (EH). In this study, the existence of EH in subjects with SCDS or LVAS was investigated using contrast-enhanced magnetic resonance imaging (MRI)., Study Design: Case series at university hospital., Methods: Seventeen ears from nine subjects who were diagnosed as having SCDS (five ears from three cases) or LVAS (12 ears from six cases) were studied. Ears were evaluated by 3-T MRI performed 4 hours after intravenous injection of gadodiamide hydrate. Imaging data concerning the degree of EH in the cochlea and the vestibule were compared with clinical symptoms and hearing levels for all ears., Results: All ears showed air-bone gaps at low frequencies on pure tone audiometry. None of the subjects with SCDS had episodes of acute sensorineural hearing loss (SNHL) or vestibular symptoms, except for one patient who complained of head vibration induced by loud noise. Conversely, five of six subjects with LVAS had episodes of acute SNHL or vestibular symptoms. Four of five ears with SCDS showed severe EH in the cochlea, and two ears showed mild EH in the vestibule. All ears with LVAS showed mild to severe EH in both the cochlea and vestibule., Conclusions: The present study demonstrated the existence of EH in ears with pathologic third window lesions, which might affect patients' auditory or vestibular symptoms., Level of Evidence: 4 Laryngoscope, 126:1446-1450, 2016., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To develop a new method to determine the presence of intracochlear ossification and/or fibrosis in cochlear implantation candidates with bilateral profound deafness following meningitis., Study Design: Diagnostic test assessment., Setting: A university hospital., Subjects and Methods: This study involved 15 ears from 13 patients with profound deafness following meningitis who underwent cochlear implantation. These ears showed normal structures, soft tissue, partial bony occlusion, and complete bony occlusion in 4, 3, 2, and 6 ears, respectively. We measured radiodensity in Hounsfield units (HU) using 0.5-mm-thick axial high-resolution computed tomography image slices at 3 different levels in the basal turn, the fenestration, and inferior and ascending segment sites, located along the electrode-insertion path. Pixel-level analysis on the DICOM viewer yielded actual computed tomography values of intracochlear soft tissues by eliminating the partial volume effect. The values were compared with the intraoperative findings., Results: Values for ossification (n = 12) ranged from +547 HU to +1137 HU; for fibrosis (n = 11), from +154 HU to +574 HU; and for fluid (n = 22), from -49 HU to +255 HU. From these values, we developed 2 presets of window width (WW) and window level (WL): (1) WW: 1800, WL: 1100 (200 HU to 2000 HU) and (2) WW: 1500, WL: 1250 (500 HU to 2000 HU). The results using these 2 presets corresponded well to the intraoperative findings., Conclusion: Our new method is easy and feasible for preoperative determination of the presence of cochlear ossification and/or fibrosis that develops following meningitis.

Objective: Administration of an aminoglycoside antibiotic and loop diuretic causes damage to hair cells in the organ of Corti, resulting in their death and the death of their corresponding spiral ganglion neurons. While this phenomenon has been studied previously, analysis of its effects in the whole cochlea has not been reported. The authors sought to evaluate the effects of a combination dose of kanamycin and furosemide in mice cochlea using an imaging system and computer analysis that allowed for nondestructive, whole-cochlea visualization., Study Design: Study using an animal model., Setting: Cochlear analysis laboratory., Subjects and Methods: Five mice received kanamycin and furosemide and 3 mice received saline. Cochleas were harvested and imaged with scanning thin-sheet laser imaging microscopy (sTSLIM) to analyze sensory cells and cochlea structures., Results: The drug-treated animals showed substantial loss of inner hair cells and complete outer hair cell loss. All treated mice showed spiral ganglion neuron loss with fewer neurons than control animals and decreased cell density in the middle turn of the cochlea. The spiral ligament and spiral limbus in the treated animals also showed a decrease in fibrocyte cell density in the middle to apical portion of the cochlea. The stria vascularis appeared normal in all animals., Conclusion: Imaging methods that allow for whole-cochlea analysis provide insight into changes that occur in the cochlea after ototoxic insult. Trends that may not be apparent in cross-section samples of the cochlea can be observed. Computer analysis of these trends allows them to be assessed accurately.

This study was designed to investigate the indication and advantages of contralateral implantation after postimplant meningitis (piM). Speech perception assessment, most comfortable levels and high-resolution computer tomography were used to monitor cochlear fibrosis/ossification and clinical changes in outcomes in 5 children affected by meningitis after a variable-time post-cochlear implantation. Ipsilateral ossification was found in 3 children, 1 of whom developed delayed contralateral ossification. These children were implanted on the contralateral side as they all showed deterioration of hearing performance. Results from the present paper and literature analysis suggest that, (1) piM can induce ipsilateral and contralateral ossification, (2) meningitis-induced cochlear ossification is more prone to develop in the presence of a normal cochlear structure and (3) contralateral implantation after piM has proven to be effective in restoring performance when a full electrode insertion is accomplished., (Copyright © 2013 S. Karger AG, Basel.)

Implantation of the ossified and dysplastic cochlea presents many unique challenges to both the surgeon and programming team. Altered embryology and physiology of these labyrinthine dysplasias may result in forms and functions unfamiliar to those casually involved with cochlear implants. Remarkable developments in diagnosis, surgical technique, electrode design, processing strategies, and programming have all contributed to the ability to successfully implant patient populations previously excluded from this life-changing intervention., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Post-meningitis cases with profound sensorineural hearing loss are known to have progressive labyrinthine ossification; such cases need to be implanted early. In our region, often a substantial amount of time is spent procuring the necessary finances for a cochlear implant; therefore, here we describe our technique of maintaining cochlear lumen patency in post-meningitis cases with early ossification, for a complete functional electrode insertion at a later date. This is a descriptive case study of a patient having post-meningitis profound deafness, with imaging studies showing early cochlear ossification, who was rehabilitated with a cochlear implant. At a tertiary referral centre, a 1-year-old child with post-meningitis bilateral profound sensorineural hearing loss was rehabilitated with cochlear implantation. The left cochlea with early ossification was stented with a customised sterile electrode to prevent scalar occlusion; 3 months later the stent was replaced with a commercial Nucleus Contour Advance implant. A complete insertion of the functional electrode array replaced the stent. Categories of auditory performance (CAP) were used to assess the outcome in our case. The pre-operative CAP score was 1 (detects environmental sounds) and the score at 15 months post implant was 6 (understands some spoken words). In post-meningitis cases with progressive cochlear ossification, stenting the cochlear lumen prevents scalar occlusion and ensures a complete insertion of a functional electrode at a later date.

Background: Radiological investigation is frequently undertaken to assess the aetiology of sensorineural hearing loss (SNHL)., Objective: To establish the CT measurements of the normal cochlea in children and to determine radiological criteria correlated with SNHL., Materials and Methods: A retrospective study of temporal bone CT performed in 159 children, age range from 3 days to 16 years between February 1999 and July 2004. A control group (n = 88) comprised children without SNHL; the SNHL group comprised 71 children. The width of the second turn of the cochlea (CW), the cochlear height (CH), and the width of the bony canal for the cochlear nerve (WCN) were measured on a reference plane containing the modiolus, the posterior semicircular canal, the footplate, and the stapes arch., Results: Width of the canal measurements or=2.5 mm supported the diagnosis of SNHL with a specificity of 97% and 91%, respectively. Cochlear width was found to be significantly smaller in the SNHL group (5.61 +/- 0.51 mm) than in the control group (5.75 +/- 0.31 mm, P < 0.02), a size <5.4 mm being highly suggestive of SNHL with a specificity of 90%. No significant variations of all measurements were found with age., Conclusion: Appropriate measurements of WCN and CW are highly correlated with SNHL.

Objective: To describe a safe and effective technique for the implantation of common cavity cochleae., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Fifteen cochleae in 9 adults and children with common cavity deformities implanted with and without the use of intraoperative fluoroscopy., Intervention(s): : Fluoroscopically assisted cochlear implantation., Main Outcome Measure(s): Rates of extracochlear placement, bending, kinking, intrameatal placement, and complications were analyzed. Numbers of electrodes inserted, number of active electrodes, and speech performance were compared between the 2 groups., Results: Half of the nonfluoroscopy group had suboptimal placement of their electrode arrays. This group also had a higher rate of complications, lower numbers of electrodes implanted, and lower number of active electrodes at follow-up. Results of speech perception testing suggest that fluoroscopically assisted implantation of the common cavity results in improved performance., Conclusion: Intraoperative fluoroscopy is an easy, safe, and effective technique that results in improved outcomes for cochlear implant patients with common cavity deformities.

Objectives/hypothesis: The present study is a long-term follow-up of speech perception outcomes and cochlear implant use in three cases of meningitis that occurred after cochlear implantation., Study Design: Case series study., Methods: Study was performed on three children implanted with different models of Clarion devices, two of them with positioner. Recognition and comprehension were assessed via the Italian adaptation of GASP (TAP) test, and phonetically balanced bi-syllabic words in open-set. High resolution computed tomography scan acquisition was performed to obtain axial coronal and oblique multiplanar reconstructions of the cochlea., Results: Two patients were affected by enlarged cochlear acqueduct and Mondini malformation the first carrying positioner. One patient had a normal cochlea, and the positioner could have been the main cause of bacterial spread. As a consequence of meningitis the child with normal cochlea and the other with enlarged vestibular acqueduct developed cochlear ossification, increased M-level and worsening of hearing outcomes. The child with Mondini malformation developed facial nerve stimulation. Contralateral implantation was performed in the first two patients., Conclusion: Bacterial meningitis occurring after cochlear implantation may induce cochlear ossification, facial nerve stimulation, and permanent or temporary loss of implant use. Planned follow-up with high resolution computed tomography and evaluation of M-levels could be useful prognostic tools in the management of these patients.

Objective: To describe our newly devised method of viewing intracochlear ossification for the purpose of minimizing bone drilling during cochlear implantation for partially ossified cochleas and to evaluate its usefulness., Study Design: Descriptive study that compares 1) conventional computed tomographic (CT) films with the newly devised CT movie and 2) preoperative evaluation using the CT movie with the findings during surgery., Setting: University Hospital., Patients: Four cochlear implantees with partial ossification of cochlea due to meningitis., Intervention: Diagnostic., Main Outcome Measures: Reconstruction of high-resolution CT images of the temporal bone was performed using a multiplanar reformat software. By rotating the cochlear cross plane from the round window niche in the direction of the inferior segment by 5 degrees, 72 images were obtained for 360-degree rotation, which were converted to a movie using QuickTime Pro software. The preoperative evaluation and intraoperative findings were compared., Results: The advantage of CT movie over sequential CT films was facility in understanding the 3-dimensional space in the cochlea. Evaluation of the extent of the ossified region on CT movie corresponded to the intraoperative findings in all 4 patients. In 1 patient, the CT movie corrected an erroneous evaluation based on conventional CT films. However, additional drilling over the extent of ossification was necessitated in another patient because of scar tissue development., Conclusion: Computed tomographic movie is considered an innovative new method for preoperative evaluation of ossified cochleas and is able to reduce human errors. However, additional drilling beyond ossified part can become necessary during surgery.

Transcranial-cerebral sonography (TCCS) is a noninvasive technique that allows clinicians to detect nanoliter (billionths of a liter) displacements of the tympanic membrane. This technique was developed to assess cerebrospinal fluid (CSF) pressure in cases of shunted hydrocephalus; it takes advantage of the CSF connection to the inner ear through the cochlear aqueduct. The movements of the tympanic membrane that are observed in TCCS are those evoked by the acoustic stapedius reflex and those spontaneous movements generated by intracranial arterial, venous, and respiratory pulses transmitted through the inner ear to the stapes and thence to the tympanic membrane. Analysis of the amplitude and direction of these displacements has enabled neurosurgeons and neurologists to estimate CSF pressures accurately in patients evaluated by TCCS. TCCS allows for applications in neurootology, particularly in those patients who present with symptoms of pulsating tinnitus, dizziness and imbalance, or hearing loss. This preliminary report describes the test and its application in a series of patients whose diagnoses included pulsating tinnitus, idiopathic intracranial hypertension, Ménière's disease, perilymphatic fistula, perilymphatic hypertension, arterial stenosis, and Arnold-Chiari syndrome. We conclude that TCCS is a valuable addition to the armamentarium of neurootologists.

Objective: With cochlear implantation, insertion of the electrodes can be hampered by cochlear obstructions. The aim of this study was to investigate the effects of these obstructions on the number of activated electrodes and postoperative speech perception., Study Design and Setting: Retrospective analysis of the operation reports and CT-scans of patients who underwent cochlear implantation, at the Department of Otorhinolaryngology of the University Medical Center of Utrecht (n = 295)., Patients and Interventions: Fifty patients with a certain degree of cochlear obstruction were included. Based on the surgical intervention to overcome these obstructions they were subdivided into first-, second- and third degree obstructions. The number of implanted and activated electrodes was determined., Main Outcome Measures: Three different speech perception tests after 12 months of cochlear implant use., Results: The number of activated electrodes significantly diminished with increasing obstruction degree (P < 0.01). This was caused by a lower amount of implanted electrodes combined with a higher amount of switched off electrodes. With a higher degree of obstruction the amount of postoperative speech perception decreased significantly (P < 0.01). This is partly explained by the lower number of activated electrodes and partly explained by the fact that a higher obstructions degree correlates with a higher degree of (retro-) cochlear pathology. Significantly more electrodes can be inserted into the severely obstructed cochlea using a Double Array Cochlear Implant., Conclusion: Cochlear obstructions pose a surgical threat and negatively influence the postoperative speech perception results. Fortunately, due to surgical techniques, first and second degree obstructed cochleas can often be bypassed. Implanting of a Double Array implant brightens the prospects of patients with third (severe) degree obstructed cochleas.

Objective: To discuss the clinical aspects and management of promontory fistula of the cochlear labyrinth., Study Design: Case report and review of the literature., Setting: University hospital, tertiary referral center., Patient, Intervention, and Results: The authors describe an unusual case of cochlear fistula localized to the promontory discovered during tympanoplasty for noncholesteatomatous chronic otitis media in a 59-year-old woman. Bone conduction was slightly impaired after operation and hearing improved after a revision myringoplasty performed for reperforation., Conclusion: Erosion of the bone of the labyrinth can also be observed in noncholesteatomatous otitis media. The presence of a fistula is not always associated with profound hearing loss. Overlying pathologic tissue can be removed without damaging the membranous labyrinth.

A 26-year-old man was admitted to our hospital with chronic left ear drainage. He had a history of meningitis when he was a child. Computed tomography (CT) of the temporal bone showed complete obliteration of the otic labyrinth by sclerotic tissue. Based on CT findings, the patient was diagnosed as labyrinthitis ossificans. Computed tomography is an appropriate method of examination for the identification of labyrinthitis ossificans and is of particular importance for the evaluation of patients before cochlear implantation.

Objective: To illustrate that a patient with a cochlear implant may be at increased risk of meningitis secondary to developmental anatomic abnormality associated with the underlying sensorineural hearing loss, as opposed to the implant itself., Methods: Case report., Results: Our 12-year-old patient has bilateral cochlear dysplasia, profound sensorineural hearing loss and no prior history of recurrent acute otitis media or meningitis. He underwent a left cochlear implant at 8 years of age and subsequently experienced three episodes of right acute otitis media and meningitis over the next 4 years. Middle ear exploration revealed a cerebrospinal fluid leak. A right radical mastoidectomy with closure of the external auditory canal, removal of the tympanic membrane, malleus, and incus, closure of the Eustachian tube, and obliteration of the mastoid and middle ear with abdominal fat has prevented further episodes., Conclusion: Meningitis in a patient with a cochlear implant is not necessarily related to the implant.

Purpose: To evaluate the prevalence of localized hypoattenuating areas in the cochlear otic capsule at temporal-bone thin-section computed tomography (CT) in pediatric patients and correlate the findings with clinical information., Materials and Methods: Temporal-bone thin-section CT images obtained in 73 patients aged 0-9 years (20 Austrian, 53 Finnish; 36 female and 37 male patients) were evaluated for the presence of localized hypoattenuating foci in the region of the fissula ante fenestram of the otic capsule. Clinical information collected for all patients was also evaluated. The data were analyzed with a logistic regression model., Results: Hypoattenuating areas in the region of the fissula ante fenestram were observed in 23 of 73 patients (32%). Hypoattenuating foci were substantially more prevalent in patients younger than 3 years than in those 3 years or older (odds ratio, 0.14; 95% CI: 0.04, 0.52; P =.001). The prevalence did not differ between sexes or according to clinical diagnosis. Only three of the 23 patients with hypoattenuating foci had clinical findings suggestive of otosclerosis, and none had osteogenesis imperfecta. After adjustment for age and sex, the finding was more prevalent among the Finnish patients (odds ratio, 5.4; 95% CI: 1.19, 24.52; P =.02) than among the Austrian patients., Conclusion: Hypoattenuating areas in the region of the fissula ante fenestram in the otic capsule at thin-section CT are prevalent among children younger than 3 years in the absence of clinical evidence of otosclerosis or osteogenesis imperfecta and appear in children up to 9 years old., (Copyright RSNA, 2004)

Optimal imaging protocols for cochlear implantation have yet to be determined. Pre-operative computed tomography (CT) and magnetic resonance image (MRI) scans are used to assess cochlear anatomy and patency, to delineate surgical access, and to aid in choice of side for implantation. However, opinion still differs as to which modality provides more information in pre-operative assessment, or if, indeed, a combination of the two is superior. The first 88 patients on the Irish National Cochlear Implant Programme (NCIP) were retrospectively studied to determine the accuracy of pre-operative CT and MRI in predicting abnormalities at the time of surgery. Correlation with surgical findings was determined in three separate groups of patients (those who had CT only, those who had MRI only, and those who had both CT and MRI performed). Of the 24 patients that had both CT and MRI performed, both modalities had a 79 per cent correlation with surgical findings. CT and MRI reports concurred in 75 per cent of cases. Specificity and negative predictive value were high (86 per cent and 90 per cent, respectively). CT alone (47 cases) correlated with surgery in 39 cases (83 per cent); MRI alone (17 cases) correlated in 15 cases (88 per cent). The findings of this study suggest that CT and MRI are effective at predicting normal inner ear anatomy, and thus at predicting the patient and the cochlea most suitable for implantation. Both modalities are useful in determining the side of implantation, thus avoiding potential surgical difficulties in cases of unilateral abnormalities. There was no significant difference between the ability of MRI and CT to detect abnormalities at the time of surgery. In this series the combination of CT and MRI has not been shown to be superior to either modality used alone, although anecdotal evidence to the contrary was noted.

Objectives: We compared the preoperative findings of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) with those observed during surgery to determine their value for cochlear implant patients., Patients and Methods: A retrospective assessment was made on preoperative HRCT and MRI scans of 124 cochlear implant patients (71 males, 53 females; mean age 15 years; range 2 to 63 years). Congenital cochlear anomalies, cochlear ossification, new bone formation in the middle ear, and structures in the internal auditory canal were evaluated and compared with intraoperative findings., Results: Cochlear anomalies detected in five patients by HRCT and MRI were confirmed intraoperatively. Cochlear ossification encountered in six patients during surgery was only demonstrated in four patients; HRCT and MRI scans only showed a narrow basal turn in the remaining two patients. During surgery, eight patients were found to have cochlear fibrosis: this finding was documented in five patients (62.5%) by MRI, and in none by HRCT scans., Conclusion: Although HRCT provides valuable information for surgical planning, its limitations may justify the additional use of MRI before cochlear implantation.

Cochlear ossification, considered until only a few years ago as a contraindication for cochlear implants (C.I.), may now be managed by means of a wide variety of surgical techniques. In cases with massive ossification, the drill-out circummodiolar technique described by Gantz et al. in 1988 and successively modified by Balkany et al. in 1997 may be adopted. The technique of electrode insertion in the scala vestibuli, perfected by Steenerson et al. in 1990, may be used when cochlear ossification has spread no further than the scala tympani. Other methods call for a groove to be drilled along the proximal tip of the basal turn of the cochlea (Cohen and Waltzman, 1993), the insertion of electrodes through the middle cranial fossa (Colletti et al., 2000), or the utilization of a double electrode array (Bredberg et al., 1997, Lenarz et al., 2001). This study reports the experience conducted at the Cochlear Implants Centre of the Otorhinolaryngoiatrics, Otological and Otoneurological Microsurgery Section of the University of Parma in a group of 15 patients who underwent C.I. in the presence of varying degrees of ossification. In 3 cases the ossification was limited to the region of the round window and a few millimetres of the scala tympani; cochleostomy was performed anteriorly and inferiorly to the anterior niche of the round window. In 11 cases (of which 3 of pediatric age), the ossification had spread to the horizontal portion of the scala tympani; in these cases, the electrodes were inserted in the scala vestibuli. The scala vestibuli was opened by drilling anteriorly to the round window and superiorly to the spiral ligament. In the only case of massively ossified cochlea, it was possible to partially insert the electrodes in a circum-modiolar tunnel. In the 12-month follow-up hearing test, the 3 patients with ossification of the round window region and the first millimetres of the scala tympani respectively averaged 61.6% in recognizing 2-syllable words and 59% in recognizing words embedded in phrases. The averages on the 12-month follow-up hearing test in the 8 adult patients who received the implant in the scala vestibuli were 80.6% in recognizing 2-syllable words and 89.1% in recognizing words in phrases. The 3 pediatric patients were classified on the Geers and Moog scale, which situated 2 of them in the 6th category of perception and 1 of them in the 4th category of perception. As regards the only case of massive cochlear ossification, the patient underwent surgery recently, and the sole follow-up available is the one conducted after only 3 months; the vowel identification average was 55%; the average on the VCV test was 31%; and the 2-syllable word recognition average was 20%.

Objective: The purpose of this study was to report hearing results obtained in a group of subjects who received scala vestibuli implantation because of cochlear ossification and to compare these results to those in patients with scala tympani implantation., Study Design: Retrospective analysis of consecutive cochlear implant procedures., Setting: Department of Otolaryngology, University of Parma., Patients: Five postlingually deafened adults with an electrode array placed into the scala vestibuli were compared with the speech performance of matched controls who had the electrode array inserted into the scala tympani., Main Outcome Measures: Measures included vowel and consonant speech identification scores, bisyllabic word and sentence speech recognition scores, and common phrases comprehension scores., Results: No significant difference was detected on speech performances between the subjects with scala vestibuli implantation and the control group., Conclusion: Scala vestibuli implantation appears to be an excellent alternative in cases in which scala tympani is found to be not patent.

The human vestibule has preserved an ancestral sound sensitivity and it has been suggested that a reflex could originate from this property underlying cervical muscle micro-contractions secondary to strong acoustic stimulation. Previous studies have established that an early component of loud sound-evoked myogenic potentials from the sternocleidomastoid muscle originate in the vestibule. This is based on findings that the response can still be obtained from patients with complete loss of cochlear and vestibular (semi-circular canal) function. Our data confirm, in a more direct way, a saccular origin of this short-latency acoustic response and verifies that a saccular acoustic response persists in the human ear. The contribution of this response to the perception of loud sounds is discussed. It is concluded that vestibular response to sound might be used to assist in the rehabilitation of deafness.

In the examination of 44 patients with primary hypoplasia of the vertebral artery (less than 2.5 mm in diameter) associated with osteochondrosis of the cervical segment of the vertebral column there have been revealed the following neuropathological manifestations of cerebral circulation insufficiency in the vertebral-basilar basin: cephalalgia, cochlear syndrome, vestibulocerebellar syndrome, vegetovascular dystonia. Ultrasound investigation of the vertebral arteries bloodflow showed a decrease in bloodflow in the intracranial length of the hypoplastic artery and an increase in the resistance to bloodflow in the extracranial segments (V2, V3). Ten patients with functional disorders in the cervical portion of the spine were exposed to orthopedic therapy directed to restoration of mobility and of topography of individual motor segments of the spinal cervical section. The studies made showed that hypoplasia of vertebral arteries can present as cerebral circulatory insufficiency in the vertebral-basilar basin, which tends to increase or reveals itself in a concurrent development of vertebrogenic reflex vasoconstriction. Restoration of the spine function (mobility) is an effective medico-prophylactic measure aimed to resolve the vertebrogenically caused spasm of the artery.

The ossified cochlea, although rare, represents a challenge for cochlear implantation. While it is no longer considered an absolute contra-indication to implantation, insertion may be technically difficult and the results may be suboptimal. Techniques which have been employed are reviewed. The new Digisonic multi-array implant, which was designed specifically for use in the ossified cochlea is described, along with the technique used for its insertion. In the first patient to be implanted with this new implant, all electrodes lie within the cochlea and are functional. The new Digisonic multi-array implant may have advantages over other solutions for the ossified cochlea.

This paper is a retrospective review of 5 patients with various degrees of cochlear ossification who were implanted with the CLARION Multi-Strategy Cochlear Implant. Preoperative computed tomography scans, intraoperative findings, surgical technique, and hearing outcomes are discussed in a case report format. Full implantation was achieved in all cases by a systematic approach that included drill-through of proximal obstruction (2 cases), scala vestibuli insertion (2 cases), and complete drill-out (1 case). The only complication was delayed wound healing in a patient with sickle cell disease, chronic active hepatitis, and steroid dependency on antimetabolite therapy. Early results show that the 4 patients with at least 3 months of experience have a mean open-set sentence recognition score of 55% and a mean open-set word recognition score of 24%. The conclusion is that implantation of the Clarion device in ossified cochleas can be successful in all degrees of ossification and can provide significant hearing benefit.

This report describes the successful implantation of the CLARION Multi-Strategy Cochlear Implant electrode in the totally ossified cochlea of a 5-year-old child via a radical mastoidectomy approach. Postoperatively, the child demonstrated responses to auditory stimuli, even though the electrode array contacted only bone and muscle graft tissue with no visible evidence of nerve fibers or cochlear lumen. Responses to sound did not begin to emerge until 10 weeks following initial stimulation and improved slowly over time. Although the child's postoperative auditory performance is more limited than that of most implanted children, she derives substantially more benefit from her implant than she did from conventional hearing aids.

A 22-year-old woman presented with severe mixed hearing loss and a flow of cerebrospinal fluid in the middle ear during stapes surgery (stapes gusher). HRCT of the temporal bones showed characteristic abnormalities of the inner ear (bulbous dilatation of the lateral portion of the internal acoustic meatus with incomplete separation from the cochlea, and widening of the first part of the facial nerve canal) described in X-linked progressive mixed deafness with stapes gusher. The evaluation of the patient's family revealed a sister with the same clinical history and identical HRCT findings, and 11 normal male relatives. This is the first report with typical findings of this entity that affects only female members of a family, suggesting another type of inheritance.