Long-Term Audiometric Follow-up of Click-Evoked Auditory Brainstem Response in Hearing-Impaired Infants: Seguimiento audiométrico a largo plazo de los potenciales evocados auditivos del tallo cerebral (PEA) en niños hipoacüAsicos

2000 ◽  
Vol 39 (3) ◽  
pp. 135-145 ◽  
Author(s):  
Ruurd Schoonhoven ◽  
Pieter J.J. Lamoré ◽  
Jan A.P.M. de Laat ◽  
Jan J. Grote
Keyword(s):  
Auditory Brainstem Response ◽  
Auditory Brainstem ◽  
Hearing Impaired ◽  
Brainstem Response

scholarly journals Audiological findings of a patient with H syndrome: case report

2021 ◽  
Vol 37 (1) ◽  
Author(s):  
Diala Hussein ◽  
Büşra Altın ◽  
Münir Demir Bajin
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Auditory Brainstem Response ◽  
Pure Tone Audiometry ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Brainstem Response ◽  
The Right

Abstract Background H syndrome is an autosomal recessive disorder caused by mutations in SLC29A3. Hyperpigmentation, hypertrichosis, hyperglycemia, and hearing loss are some characteristics of this disorder, and it has a prevalence of < 1/1000. The aim of this report is to spread awareness among otologists, audiologists, and pediatricians about this syndrome and its audiological features. Case presentation An 8-year-old male with a diagnosed H syndrome registered to our clinic with a complaint of hearing loss. The patient was diagnosed with hearing loss in a different clinic using only the air-conducted click auditory brainstem response test which showed wave V at 60 dB nHL for the right ear and at 80 dB nHL for the left ear. The initially performed pure tone audiometry (PTA) test in our clinic revealed a bilateral asymmetric hearing loss with a moderate sensorineural hearing loss in the right ear and a profound mixed hearing loss in the left ear. The performed air conducted click auditory brainstem response (ABR) result showed wave V at 55 dB nHL for the right ear and at 70 dB nHL for the left ear. Then, the repeated PTA test revealed a mild-severe sensorineural sloping hearing loss in the right ear and a severe sensorineural hearing loss in the left ear. Conclusion Although hearing thresholds in H syndrome could be within normal limits in some patients, sensorineural hearing loss is an important characteristic feature for this syndrome. Sensorineural hearing loss could be progressive or of sudden onset and ranges from mild to profound. Thus, it must be taken into consideration to apply the audiological follow-up regularly and paying attention to the patient’s complaints; also, a regular follow-up for language development of children with H syndrome and for the hearing aids is advised.

Long-term course of Landau-Kleffner syndrome:

1999 ◽  
Vol 16 (1) ◽  
Author(s):  
Carla G. Matas ◽  
Renata A. Leite ◽  
Letícia L. Mansur ◽  
Laura M.F.F. Guilhoto ◽  
Maria Luiza G. Manreza
Keyword(s):  
Auditory Brainstem Response ◽  
Epileptic Seizures ◽  
Auditory Brainstem ◽  
Cognitive Domain ◽  
Speech Acquisition ◽  
Middle Latency Response ◽  
Brainstem Response ◽  
Latency Response ◽  
Middle Latency

Introduction. Landau-Kleffner Syndrome is characterized by normal speech acquisition followed by epileptic seizures, receptive and expressive language deterioration coupled with agnosia for non-verbal sounds, having variable long-term evolution. Case Report. It is described neurophysiologic and acoustic findings in a patient with Landau-Kleffner Syndrome, and correlate these with the results of a language evaluation carriedout 7 years after the acute phase. It is performed Electroencephalography, Immitance Measurements, Basic Audiometry, Auditory Brainstem Response, Middle Latency Response, and P300. Language was evaluated by Boston Diagnostic Aphasia Examination. Electroencephalography was normal and audiologic evaluation revealed normal Immitance Measurements, Basic Audiometry and Auditory Brainstem Response values. Anelectrode effect was present in the left hemisphere in Middle Latency Response, and bilateral P300 latencies delayed on the right. Language evaluation showed severe receptive and expressive impairment, severe phonemic substitutions, which had an impact on social and academic levels. There were contextual and gestual non-verbal compensations, evidencing intellectual and cognitive domain preservation. Conclusion. This case illustrates the specific cerebral areas that can be damaged in patients with Landau-Kleffner Syndrome and which are demonstrable by clinical evaluation and proper neurophysiology studies, showing the importance of neurological, audiological, electrophysiological and language exams in a longitudinal follow up.

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