Central or combined central and peripheral hearing loss at an early born child

Central hearing loss is caused by a dysfunction of the auditory pathway and/or the auditory cortex whereas sensorineural hearing loss mostly is due to failure of the outer hair cells of the inner ear. We present the case of a child with central or combined hearing loss.

Case Report: A 5-year-old child with a complex developmental disorder and inconsistent reactions to acoustical stimuli presented. Early born child 27+6 weeks of gestation, status after (s.a.) infant respiratory distress syndrome, s.a. intraventricular bleeding with involvement of the parenchyma, s.a. posthaemorrhagic hydrocephalus, s.a. implantation of a rickham-reservoir and a VP-shunt, PDA, VSD, PFO, homozygote MTHFR mutation, s.a. suboccipital trepanation to remove a cyst in the isolated fourth ventricle.

ORL examination was regular, tympanogram was type A. Pure tone (free-field audiometry) showed a doubtable hearing threshold of 60 to 80 dB; speech audiometry was not possible. TEOAE were constantly irreproducible. Additional hearing tests with BERA (click and NB-Chirp) and ASSR demonstrated reproducible auditory brain stem responses above stimulation levels of 100 dB SPL. The CERA only showed low inconsistent responses. Language development showed limitations in all fields.

A recent MRI confirmed an extension of the intravenous. Ventricle at status after windowing a captured IV. Ventricle and expulsion of the vestibulocochlear nerve on both sides by the flocculus cerebelli, on the right side less than on the left side and compressed brainstem and tectum.

Results of electrophysiological, acoustical, and psychoacoustical hearing tests are inconsistent. Synopsis of the results of audiometric examinations and MRI leads to the diagnosis of a central hearing loss due to a compression of the pathway. Irreproducible otoacustic emissions allude to an inner ear damage or disturbance of outer hair cell control at the brainstem. After an adaption of bilateral hearing aids and speech therapy, an improvement of the hearing and speech was observed. However, compensation of the hearing loss was not sufficient.