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Objectives Even 250 years after Beethoven’s birth, the irrevocable cause of his hearing impairment remains unclear despite multiple publications by different professional groups. This study aimed to analyse the development of the most likely aetiologies during the last 100 years by a systematic review of the relevant medical literature. Methods A systematic review of medical literature in PubMed®, PubMed Central®, and Web of Science® for the period 1920–2020 was conducted. Medical publications between 1920 and 1935 were additionally searched manually by review of reference lists. Studies were eligible when a statement regarding the most likely aetiology of the hearing loss of Beethoven was the aimed objective of the publication. Results 48 publications were included. The following aetiologies were supposed: otosclerosis (n = 10), syphilis (n = 9), Paget’s disease (n = 6), neural deafness (n = 5), immunopathy with inflammatory bowel disease, neural deafness with otosclerosis, sarcoidosis or lead intoxication (n = 2), and systemic lupus erythematosus, trauma, labyrinthitis or inner ear disease (n = 1). There is an ongoing effort with a mean publication frequency in this topic of 0.48/year. From 1920 to 1970, otolaryngologists were the group with the highest interest in this field (67%), whereas since 1971 most authors have belonged to non-otolaryngologic subspecialities (81%). Conclusion Over the past 100 years, otosclerosis and syphilis were predominantly supposed to be the underlying causes. The hypothesis of syphilis—although rejected for a long time—has had a remarkable revival during the past 20 years. Regarding the outcome following therapeutic intervention by cochlear implantation, the differential diagnosis of neural deafness would be relevant today.

The objective of our study was to review retrospectively the clinical, radiological and therapeutic findings in 62 adults with acute mastoiditis treated at the ENT Department of Ferrara from 1992 to 2010. 62 adult cases fulfilled the following inclusion criteria: otoscopical evidence of co-existent or recent otitis media; postauricular swelling, erythema or tenderness; protrusion of the auricle; fever and/or significant radiological findings of mastoiditis. Conservative treatment comprehended antibiotic ± ventilation tube. Surgical procedures comprehended mastoidectomy or mastoido-tympanoplasty. The incidence of adult's mastoiditis in our district (0.99 cases/year/100.000 inhabitants) has maintained quite stable during the considered 19-year period. The typical clinical presentation was observed in 48% of cases. Complications were meningitis (15 cases), meningo-encephalitis (1), meningitis associated with lateral sinus thrombosis (1), facial nerve paralysis (11), and labyrinthitis (8). In all cases except one, the facial palsy recovered completely and no mortality was observed due to these complications. Complete cure was obtained with conservative treatment in 69% of uncomplicated cases and in 24% of patients with intracranial complications. Mastoiditis in adults may present as the acute classical form, as well as latent forms which often have prolonged and insidious development followed by a rapid clinical deterioration. Clinical features are frequently atypical, while incidence of meningitis and other complications is still high particularly in the most elderly. Thus, great care is required from clinicians to make an early diagnosis in order to promote adequate treatment.

The literature about bony defects in the semicircular canal system is highly inconsistent. Therefore, we analyzed a series of 700 high-resolution multislice CT examinations of the temporal bone for semicircular canal dehiscencies. An unselected group of ENT patients with different clinical symptoms and variable age was chosen. We found semicircular canal dehiscence in 9.6% of temporal bones, superior semicircular canal was affected mostly (8%), less common posterior semicircular canal (1.2%); only in 3 cases (0.4%), lateral semicircular canal showed dehiscence. In 60% of SSC dehiscence, we registered bilateral manifestation. The so-called "third mobile window" in semicircular canal dehiscence causes a great variety of clinical symptoms like vertigo, nystagmus, oscillopsies, hearing loss, tinnitus and autophonia. Comparison with anatomic studies shows that CT examination implies the risk of considerable overestimation; this fact emphasizes the important role of clinical and neurophysiological testing.

This study was designed to compare tympanic membrane (TM) and middle ear (ME) pathologies of temporal bones from children and adults with purulent otitis media (POM). Thirty-four temporal bones were used from 22 subjects ages 2 days to 76 years with histopathologic evidence of POM. There were 55 age-matched controls. Histopathologic findings of the TM and ME in children and adults with POM were compared. Clinical histories and the presence of complications were recorded. The incidence of POM was more common in male children than in females. There was a significant increase in the thickness of the posterosuperior and posteroinferior quadrants in children with POM compared to non-OM children. In adults with POM, there was a significant decrease in the thickness of the posteroinferior and anteroinferior quadrants compared to non-OM adults. Children with POM showed a significant increase in the anterioinferior and posteroinferior quadrants and the umbo compared to adults with POM. Pathology of the TM and ME occurred in adults and children, but severity was greater in children. Residual mesenchyme was frequently observed in temporal bones of children. Serious complications such as labyrinthtis and meningitis were observed more frequently in children. All cases with meningitis had labyrinthitis, previous histories of otitis media and had been treated with antibiotics. Although POM occurs in both children and adults, pathologic changes of the middle ear are more severe, and complications (labyrinthitis and meningitis) occur more often in children. Our findings suggest the need to monitor children carefully under the age of 2 years who have POM.

Although gadolinium-enhanced magnetic resonance imaging (Gd-MRI) has been used to indicate the presence of a subclinical labyrinthitis in patients with idiopathic sudden sensorineural hearing loss (ISSHL), its sensitivity in daily clinical practice is unknown. We describe Gd-MRI findings in 27 ISSHL patients taking part in a prospective multicenter clinical trial. MRI findings were related to the severity of the hearing loss, vestibular involvement and the time interval between the occurrence of ISSHL and imaging. Pathological enhancement of the cochlea indicating a labyrinthitis was found in one patient with ISSHL. In 26 cases, no pathological enhancement could be established. Study results indicate that the present sensitivity of Gd-MRI is low for detecting subclinical viral labyrinthitis in patients with ISSHL. To improve the sensitivity of MRI in ISSHL, we recommend that imaging is performed as early as possible, preferably before treatment is started.

The cochlear aqueduct is a bony channel which contains the fibrous periotic duct and connects the perilymphatic space of the basal turn of the cochlea with the subarachnoid space of the posterior cranial cavity. Previous histological studies suggested that patency depended on age, whereas a more recent study showed no statistical correlation between age and patency. To clarify patency in pediatric cochlear aqueducts, we selected 21 temporal bones from 12 infants and children, varying in age from birth to 9 years, in which the cochlear aqueduct was fully visible on one histological section. Photographs were taken for documentation and the length and width of the orifice of the external aperture of the aqueduct at the scala tympani were measured and followed to the internal aperture at the subarachnoid space. The lumen of the duct was examined for mononucleated cells, blood cells and fibrous tissue. Measurements revealed that the mean length of the cochlear aqueduct was 4.6 mm (range, 2.4-10.7 mm), mean width of the external aperture was 484 microm (range, 225-869 microm), and mean width of the internal aperture was 1293 microm (range, 699-2344 microm). The mean diameter of the narrowest part (isthmus) was 151 microm (range, 75-244 microm). In all temporal bones the cochlear aqueduct was patent, with one exception. This latter temporal bone was from a 2-month-old girl with multiple intralabyrinthine anomalies, with the missing cochlear aqueduct believed to be due to an aplasia. Our results support prior measurements of the cochlear aqueduct and demonstrate a short and patent cochlear aqueduct in newborns. With growth, a significant increasing length of the duct was found.

We report our experiences in managing a patient with cholesteatoma complicated by meningitis, labyrinthitis and facial nerve palsy. The antero-inferior half of the tympanum was aerated but the postero-superior portion of the tympanic membrane was tightly adherent to the promontry mucosa. An attic perforation was present at the back of the malleolar head. High-resolution computed tomography also uncovered a fistula in the lateral semicircular canal. Surgical exploration of the middle ear cavity demonstrated that both the vestibule and cochlea were filled with cholesteatoma, and the cholesteatoma extended into the internal auditory meatus through the lateral semi-circular canal fistula. The cholesteatoma was removed by opening the vestibule and cochlea with a preservation of the facial nerve. Post-operatively, an incomplete facial palsy remained, but has improved slowly. There is no sign of recurrence to date after a 3-year period of observation.