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Updated: Aug 4, 2026
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
[Congenital adrenal hypoplasia and hearing loss. A case report]
A Liotta1, C Maggio, L Casimiro
1Istituto di Pediatria, Università degli Studi, Palermo.
Insights
Early diagnosis of X-linked congenital adrenal hypoplasia is crucial. Prolonged follow-up can reveal associated conditions, such as the mild hearing loss identified in this newborn.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- X-linked congenital adrenal hypoplasia (X-linked CAH) is a rare genetic disorder affecting adrenal gland function.
- Early diagnosis and management are critical for patient outcomes.
- Associated conditions can occur and require timely identification.
Abstract:
We report on the diagnostics, the therapeutics and the follow-up (to 3 years and 5 months) of a newborn affected by X-linked congenital adrenal hypoplasia. After the beginning of substitute hormonal therapy, the patient underwent periodical clinical examinations, with particular attention to the growth, and laboratory tests, which monitored the hormonal pattern. This experience points out the diagnostic role of low maternal levels of urinary estriol during pregnancy and the importance of a prolonged follow-up, so as to discover associated pathologies as early as possible. In our case, in fact, the study of auditory brainstem responses enabled us to recognize a mild transmission hearing loss at the early age of 8 months.
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