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We report a rare case of isolated malleus dislocation into the external auditory canal with lateralized intact tympanic membrane following a head trauma. The patient was a 63-year-old woman who presented at the outpatient department of our institute with hearing loss of 10 years' duration after a bicycle accident. During physical examination of the patient, total dislocation of the malleus-like bony structure into the external auditory canal on the right side was observed. In the computed tomography scan, an isolated malleus dislocation with intact incus-stapes articulation was identified. The patient was successfully treated with endoscopic exploratory tympanotomy and partial ossicular replacement prosthesis. The isolated malleus dislocation can rarely occur after trauma. A careful diagnostic step through history, physical examination, and temporal bone computed tomography scan are needed to confirm this rare condition. [ABSTRACT FROM AUTHOR]

Objective: Tinnitus is a common symptom of idiopathic sudden sensorineural hearing loss (ISSHL). This study aimed to investigate the characteristics and outcomes of acute tinnitus in patients with ISSHL. Methods: A total of 59 patients with ISSHL and acute tinnitus were enrolled. All patients underwent audiological assessment, tinnitus matching for loudness and frequency, and steroid treatment for sudden hearing loss. Tinnitus‐related distress was assessed using the tinnitus handicap inventory (THI) questionnaire. The outcomes of hearing recovery and tinnitus remission were investigated retrospectively. Results: The loudness and pitch of acute tinnitus were 63.2 ± 22.4 dB HL and 2010.63 ± 2368.99 Hz, respectively. Complete and partial recovery rates of acute tinnitus in ISSHL patients were 32.2% and 39% according to the THI scores. The group with complete recovery of hearing showed significantly greater improvement in tinnitus distress than the group with no improvement in hearing. The loudness and pitch of tinnitus did not correlate with tinnitus remission. Conclusion: Hearing recovery is more important for tinnitus remission than the psychoacoustic characteristics of acute tinnitus. Timely identification and proper treatment of hearing loss are important to improve tinnitus remission in patients with ISSHL. Level of Evidence: Level 4. [ABSTRACT FROM AUTHOR]

Background: Many mutations in the α-tectorin gene (TECTA) have been reported to cause non-syndromic hearing loss (NSHL) in either a dominant or recessive inheritance pattern. Among the identified TECTA mutations, H1400Y has been associated with NSHL in two independent studies. However, its exact role in contributing to genetic hearing loss remains elusive. Case presentation: We herein report the whole-exome sequencing of a proband presenting with prelingual, non-progressive, mild-to-moderate hearing loss in a simplex family. By using trio-based whole-exome sequencing, we found two heterozygous mutations of R1890C and H1400Y in the ZP and ZA domains of TECTA, respectively. R1890C, previously reported as a pathogenic autosomal dominant mutation of genetic hearing loss, was found to be inherited in a de novo pattern, causing hearing loss in the proband. By contrast, H1400Y was not segregated in this family, and one family member with normal hearing also carried the H1400Y mutation. Conclusion: According to the hearing loss-specific American College of Medical Genetics and Genomics (ACMG) guidelines, we conclude that H1400Y should be disqualified as a cause of genetic hearing loss. True pathogenic variants causing genetic hearing loss should be more deliberately reported in accordance with ACMG guidelines. [ABSTRACT FROM AUTHOR]