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Simple Summary: Squamous cell carcinoma (SCC) of the external auditory canal (EAC) is a rare malignancy, and its disease-free survival (DFS) is strongly associated with surgical margins and tumor classification. Early-stage EAC SCC has higher survival rates than advanced-stage EAC SCC, suggesting that less aggressive treatment strategy might be sufficient. However, the additional benefits of post-operative radiotherapy (PORT) for early-stage EAC SCC that has been completely resected are unclear. Our retrospective study aimed to evaluate whether PORT offers additional benefits for such cases. Our results showed no significant difference in 5-year DFS between patients treated with PORT and those without PORT. EAC SCC treated with PORT more frequently exhibited perineural and angioinvasive growth compared to those without PORT. Thirty-eight percent of the patients undergoing PORT experienced side effects of radiotherapy. These findings suggest that PORT should be reserved for selected cases with high-risk features to minimize side effects and preserve quality of life. Background: There is no consensus regarding the indication for postoperative radiotherapy (PORT) for T1- and T2-classified squamous cell carcinoma (SCC) of the external auditory canal (EAC) even with negative surgical margins. This study aimed to evaluate whether PORT provides additional benefits for these cases. Methods: We collected retrospective data from fourteen international hospitals, including resected pT1- and pT2-classified EAC SCC with negative surgical margins. Results: A total of 112 early-stage radically resected EAC SCC were included, with 48 patients receiving PORT. The 5-year DFS of T1- and T2-classified EAC SCC treated with PORT was not statistically significantly different (92.9% and 76.9%, respectively) compared to the group treated without PORT (100% and 90.9%, respectively; p-values of 0.999 and 0.526, respectively). EAC SCC treated with PORT more frequently exhibited perineural and angioinvasive growth. Eighteen patients experienced side effects related to radiotherapy, of which one patient developed osteoradionecrosis. Conclusions: Our study suggests that PORT for early-stage radically resected EAC SCC should only be considered in selected cases with perineural, infiltrative growth or angioinvasive growth, and with a close margin. This approach helps mitigate the negative impact on quality of life and the risk of side effects associated with radiotherapy. [ABSTRACT FROM AUTHOR]

Objectives: This is a report of the audiological and vestibular characteristics of a Dutch DFNA9 family with a novel mutation, 1109T, in the LCCL domain of COCH. Methods: From the family with the novel 1109T COCH mutation, audiometric data were collected and analyzed longitudinally. Results were compared to those obtained in previously identified P51S, G88E, and G87W COCH mutation carriers. Special attention was also given to a comparison of age-related features such as progressive hearing loss and vestibular impairment. Results: A novel mutation (1109T) in COCH segregates with hearing impairment and vestibular dysfunction in the present family. Pure tone thresholds, phoneme recognition scores, and vestibular responses of the 1109T mutation carriers were essentially similar to those previously established in P51S, G87W, and G88E mutation carriers. Deterioration of hearing in the 11091 mutation carriers started at 43 years of age, and vestibular function deteriorated at least 7 years later. Conclusions: The phenotype associated with the novel COCH (1109T) mutation is largely similar to that associated with P51S and G88E mutation carriers. However, subtle differences in terms of onset age and rate of progression seem to exist. [ABSTRACT FROM AUTHOR]

Importance: The ideal surgical treatment of cholesteatoma has been subject to discussion for years because both traditional surgical techniques (canal wall down [CWD] and canal wall up [CWU] tympanoplasty) have their own advantages and disadvantages. A more recently propagated surgical approach, to combine the CWD or CWU tympanoplasty technique with obliteration of the mastoid and epitympanum, is showing promising results., Objective: To systematically review the literature on recurrent and residual cholesteatoma rates after single-stage CWU and CWD tympanoplasty with mastoid obliteration., Evidence Review: A systematic search of literature was performed to identify relevant publications in multiple electronic databases. The initial search was conducted in December 2016 and was updated in July 2017. Each study was reviewed by 2 independent reviewers on predetermined eligibility criteria. The methodological quality was determined using the methodological index for nonrandomized studies (MINORS) scale, and the relevance to the current topic was determined using a 4-criterion checklist., Findings: The searches identified a total of 336 potentially relevant publications; 190 articles were excluded based on title and abstract. The full-text articles of the remaining 146 citations were assessed for eligibility, resulting in 22 articles. After assessing these remaining articles for methodological quality and relevance to the current topic, another 8 studies were excluded, and a total of 13 studies (1534 patients) were included. Of the 1534 patients who underwent CWD or CWU tympanoplasty with mastoid obliteration, the rate of recurrent disease was 4.6%, and the rate of residual disease was 5.4%. In CWU tympanoplasty with mastoid obliteration, these rates were 0.28% and 4.2%, respectively and in CWD tympanoplasty with mastoid obliteration, 5.9% and 5.8%, respectively., Conclusions and Relevance: We show the recurrent and residual disease rates after either CWU or CWD tympanoplasty with mastoid obliteration to be qualitatively similar to, if not better than, previously reported rates of for nonobliterative techniques. In this study, the lowest recurrent and residual rates were reported when combining the CWU tympanoplasty with mastoid obliteration, on average 0.28 and 4.2%, respectively.

Objective: To report on the audiometric characteristics of a large Dutch family linked to DFNA15 with a novel mutation (p.L289F) in POU4F3 (OMIM 602460)., Design: Clinical investigation., Setting: Tertiary referral center., Patients: Family members from a large 5-generation pedigree with sensorineural hearing impairment segregating as an autosomal dominant trait., Main Outcome Measures: Cross-sectional and longitudinal analyses of pure-tone audiometric data, and cross-sectional analyses of speech audiometry data., Results: Overall, a flat to gently downsloping audiometric configuration was observed with a progression rate of approximately 0.8 dB/y across most frequencies. Speech recognition scores remained fairly good in relation to age and hearing level compared with a group of patients with presbycusis. Interindividual variability was observed in terms of subjective onset age and audiometric configuration. Two mutation carriers, who reported vestibular symptoms, underwent vestibular examination and showed hypofunction of the vestibular labyrinth., Conclusions: The audiometric phenotype of the Dutch family linked to DFNA15 with a novel mutation in POU4F3 is comparable to that observed in the original Israeli family linked to DFNA15. Relatively good speech recognition scores suggest outer hair cell involvement. DFNA15 may represent a cochleovestibular disorder.