[Selection of cochlear electrode array implantation lengths and outcomes in patients with bilateral sensorineural hearing loss].

Objective: To perform a comprehensive comparison on the therapeutic effects of unilateral 31.5 mm and 28 mm cochlear implantation (CI) on the post-operative hearing rehabilitation outcomes, including hearing threshold, speech recognition and quality of life, in patients with bilateral sensorineural hearing loss. Methods: A total of 26 patients [12 males, 14 females, aged 19-71 (43±16) years] diagnosed with bilateral severe-to-profound sensorineural hearing loss at Peking Union Medical College Hospital from April 2018 to August 2019 were included. Patients underwent temporal bone high resolution CT (HRCT), based on which the electrode lengths were calculated using OTOPLAN. Eleven and fifteen ears were implanted with MED-EL Flex 31.5 mm and Flex 28 mm electrode arrays respectively, via round window approach under minimally invasive surgery. The patients were followed up regularly for up to 2 years. At each follow-up, aided hearing threshold, speech recognition in quiet and noise, and Nijmegen Cochlear Implantation Questionnaire (NICQ) scores were evaluated and compared. Results: Post-operative hearing thresholds were (46.5±3.4) dB and (48.5±2.2) dB in patients implanted with MED-EL Flex 31.5 mm and Flex 28 mm electrode arrays, respectively, with no statistically significant difference (P=0.074). Both hearing thresholds and speech recognition demonstrated significant post-operative improvement compared with pre-operative results. Hearing thresholds after 1-year post-operation were (32.1±1.2) dB and (32.5±0.9) dB, respectively (P=0.355). Patients implanted with Flex 31.5 mm electrode scored significantly higher at speech recognition under 65 dB sound pressure level (SPL) at most of the follow-ups (All P<0.05). Speech recognition in noise (S/N=10 dB) was also improved in patients implanted with Flex 31.5 mm electrode. All sub-divisions of the NICQ demonstrated significant post-operative improvement, and no significant difference between the 2 groups was observed apart from the"self-confidence"sub-division. Conclusions: Selection of MED-EL Flex 31.5 mm and 28 mm implantation based on pre-operative OTOPLAN evaluation can both bring significant improvements to patients' hearing and quality of life. Flex 31.5 mm electrode can potentially provide better speech recognition within a certain period after surgery.

[IgG4-associated diseases mainly with auricular symptoms: a case report and literature review].

Summary To analyze the clinical features of IgG4-related diseases mainly with otologic manifestations and investigate diagnosis and treatment of IgG4-related diseases. We report the clinical course and diagnosis and treatment process of a case of IgG4-related disease misdiagnosed as chronic suppurative otitis media. After looking through relevant literatures, we review 22 cases of IgG4-related diseases mainly with otologic manifestations, and summarize their epidemiological characteristics, clinical manifestations, histopathologic features, radiologic features, and diagnostic and therapeutic criteria. IgG4-related diseases mainly with otologic manifestations are usually clinically characterized by atypical symptoms such as otalgia, tinnitus, aural fullness, otorrhea and progressive or fluctuating hearing loss, together with nausea, vomiting, and dizziness. The three central histopathologic features are dense lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. The exact diagnosis of IgG4-related diseases requires a combination of clinical manifestations, serology, histopathology and radiologic results. Glucocorticoids are currently the first-line approach for IgG4-related diseases. Otologic manifestations involved in IgG4-related diseases are poorly specific, which is likely to result in more misdiagnosis and treatment delay. Current golden standard for diagnosing IgG4-related diseases is the identification of characteristic histopathology. In addition, immunohistochemistry can help clinicians diagnose IgG4-related diseases as early as possible. Otologists should strengthen their understanding of otologic manifestations of IgG4-related diseases. Early diagnosis and treatments are the key factors in prognosis.

[Outcome of cochlear implantation in a patient with Chiari malformation type â who presented with hearing loss].

Summary Chiari malformation type Ⅰ（CMI） is a disorder characterized by tonsilla cerebelli herniating into an underdeveloped posterior cranial fossa, hearing loss is often covered by more striking neurological symptoms. Hearing loss in this syndrome is not specific in terms of gender side, degree, age of onset, and progression. The hearing improvement after posterior fossa decompression is controversial on the basis of literature, while satisfactory result was obtained after cochlear implantation in the patient reported here, who was diagnosed as CMI with hearing loss as the main symptom. Therefore, after ensuring the integrity of the auditory pathway, cochlear implantation may be considered in CMI patients with bilateral severe or profound without other severe neurological symptoms.