Selective inner hair cell loss in premature infants and cochlea pathological patterns from neonatal intensive care unit autopsies

Abstract

Background: Deafness and handicapping sensorineural hearing impairment occur frequently in neonatal intensive care unit survivors for unknown reasons.

Patients and methods: Hearing was tested early and repeatedly in neonatal intensive care unit patients with an auditory brainstem response (ABR) screener. The temporal bones of 15 nonsurvivors (30 ears) were fixed promptly (average, 5 hours) after death for histological evaluation.

Results: Among these patients, 12 failed the ABR screen bilaterally, 1 passed unilaterally, and 2 passed bilaterally. Cochlear histopathologic conditions that could contribute to hearing loss included bilateral selective outer hair cell loss in 2 patients, bilateral selective inner hair cell loss in 3 (all premature), and a combination of both outer and inner hair cell loss in 2. Other hair cell abnormalities were noted; the 2 infants who had passed the ABR screen demonstrated normal histological features. Neuronal counts were normal.

Conclusions: Auditory brainstem response failure among these neonatal intensive care unit infants who died was extremely common in part owing to an unexpected histological alteration, selective inner hair cell loss among premature newborns, that should be detectable uniquely by the ABR testing method. Additional histological patterns suggest more than one cause for neonatal intensive care unit hearing loss. Hair cell loss patterns seem frequently compatible with in utero damage.