In the present patients, Alström Syndrome began with a neurosensory hearing loss in early childhood that progressed to a profound loss in ten to twenty years. The auditory lesions were cochlear in origen according to the otoacoustic emissions and auditory brainstem responses.

Introduction  The bone-anchored hearing aid (BAHA) is a bone conduction system that transmits the sound directly to the inner ear by surpassing the skin impedance and the subcutaneous tissue. It is indicated for patients with mixed, conductive and unilateral sensorineural hearing loss who did not benefit from conventional hearing aids (HAs). Although the benefits from BAHA are well demonstrated internationally, this field still lacks studies in Brazil. Objective  To assess the auditory rehabilitation process in BAHA users through audiological, speech perception and tinnitus aspects. Methods  Individuals with hearing loss were assessed before and after the implantation. The participants were subjected to pure tone audiometry in free field, functional gain audiometry, speech perception tests, tinnitus handicap inventory (THI) in open format, and to the visual analog scale (VAS). Results  It was found that the participants benefited from the use of BAHA. The difference in the performance of the participants before and after the BAHA surgery was significant in terms of hearing acuity. There was no statistically significant difference in the speech perception tests. The tinnitus assessment showed that 80% of the participants scored slight tinnitus severity in THI after using a BAHA. Eighty percent of the participants classified their tinnitus as absent to mild in the VAS after the surgery. Conclusion  Based on the results of the current study, we can conclude that the participants improved both the auditory perception and the tinnitus handicap.

Introduction:In order to have a better knowledge about the development of language skills in cochlear implant (CI) users it is necessary to evaluate the speech of these subjects using appropriate tests. The ABFW-Child Language Test is a tool that relates to various areas: phonology, vocabulary, fluency and pragmatics, and that can be used with cochlear implant users for the purposes of this study.Objective: To evaluate the vocabulary of children who are cochlear implant users. Method: This study evaluated 16 children who had CI surgery between 08 and 52 months of age. The subjects were divided in five groups according to the age at the time of the CI activation, as follows: Group 1: 3 children implanted unilaterally with hearing age between 12 and 23 months of age; Group 2: children implanted bilaterally with hearing age between 12 months and 23 months of age; Group 3: 3 children implanted unilaterally with hearing age between 24 and 35 months of age; Group 4: 3 children implanted unilaterally with hearing age between 36 and 47 months of age and Group 5: 4 children implanted unilaterally with hearing age between 48 years and 59 months of age. Results: In the vocabulary test, when comparing CI users considering the auditory brain development age and the chronological age of normal hearing children, all CI users performed better than the standards for normal hearing children. When comparing children with unilateral and bilateral CI, the bilateral cochlear implant users showed better results. When comparing test performance considering the chronological age, normal hearing children performed better, but the results were similar in CI users that systematically use the device and attend auditory rehabilitation sessions.Conclusion: The vocabulary of CI users in the present study was similar to normal hearing children's vocabulary. The vocabulary test proved to be an effective measure to observe the gaps in development and helped the conduction of speech therapy in order to achieve the full development of speech and language skills of these children.

The electrically evoked compound action potential measurement using neural response telemetry in children with bilateral cochlear implants during the first year of follow-up was effective in demonstrating the synchronized bilateral development of the peripheral auditory pathways in the studied population.

Within the selected eight ears, six (75%) showed presence of otoacoustic emissions test in isolated frequencies and two (25%) ears had otoacoustic emissions test even in the presence of the isolated frequencies. It was found that 26.7% of the ears tested presented results that are compatible with Auditory Neuropathy Spectrum Disorder. Conclusion:The results of this study have identified characteristics related to ANSD in 26.7% of ears tested, 6 participants with age over 10 years old, showing that misdiagnosis in hearing loss patients is still very frequent.

In our study, the vocabulary of children using CI is similar to the vocabulary of hearing children.