Selective Inner Hair Cell Loss in Premature Infants and Cochlea Pathological Patterns From Neonatal Intensive Care Unit Autopsies | Critical Care Medicine | JAMA Otolaryngology–Head & Neck Surgery | JAMA Network
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Schulman-Galambos  CGalambos  R Brain stem evoked response audiometry in newborn hearing screening.  Arch Otolaryngol.1979;105:86-90.Google Scholar
Sanders  RDurieux-Smith  AHyde  MJacobson  JKileny  PMurnane  O Incidence of hearing loss in high risk and intensive care nursery infants.  J Otolaryngol Suppl.1985;14:28-33.Google Scholar
Bergman  IHirsch  RPFria  TJShapiro  SMHolzman  IPainter  MJ Cause of hearing loss in high-risk premature infants.  J Pediatr.1985;106:95-101.Google Scholar
Borg  E Perinatal asphyxia, hypoxia, ischemia and hearing loss: an overview.  Scand Audiol.1997;26:77-91Google Scholar
Robertson  CMCheung  PYHaluschak  MMElliot  CALeonard  NJfor the Western Canada ECMO Follow-up Group High prevalence of sensorineural hearing loss among survivors of neonatal congenital diaphragmatic hernia.  Am J Otol.1998;19:730-736.Google Scholar
Abramovich  SJGregory  SSlemich  MStewart  A Hearing loss in very low birth weight infants treated with neonatal intensive care.  Arch Dis Child.1979;54:421-426.Google Scholar
Kramer  SJVertes  DRCondon  M Auditory brainstem responses and clinical follow-up of high-risk infants.  Pediatrics.1989;83:385-392.Google Scholar
Eavey  RDBertero  MdThornton  AR  et al Failure to clinically predict NICU hearing loss.  Clin Pediatr (Phila).1995;34:138-145.Google Scholar
Eavey  RDPinto  LEThornton  ARHerrmann  BSdo Carmo Bertero  MSaenz  A Early hearing testing of still critically ill neonates.  Arch Otolaryngol Head Neck Surg.1996;122:289-293.Google Scholar
Schuknecht  HF Techniques for the study of cochlear function and pathology in experimental animals.  Arch Otolaryngol.1953;58:377-397.Google Scholar
Greenwood  DD Critical bandwidths and the frequency coordinates of the basilar membrane.  J Acoust Soc.1961;33:1344-1356.Google Scholar
Otte  JSchuknecht  HFKerr  AG Ganglion cell populations in normal and pathological human cochlea: implications for cochlear implantation.  Laryngoscope.1978;88:1231-1246.Google Scholar
Schuknecht  HF Pathology of Ear. 2nd ed. Philadelphia, Pa: Lea & Febiger, 1993.
Bachor  EKarmody  C Hydrocephalus and the status of endolymphtic membranes in temporal bones of children.  In: Ernst  R, Marchbanks  M,Samii  M, eds. Intracranial and Intralabyrinthine Fluids: Basic Aspects and Clincial Applications. Berlin, Germany: Springer Verlag; 1996:93-103. Google Scholar
Drack  AV Preventing blindness in premature infants.  N Engl J Med.1998;338:1620-1621.Google Scholar
Takeno  SHarrison  RVIbrahim  DWake  MMount  RJ Cochlear function after selective inner hair cell degeneration induced by carboplatin.  Hear Res.1994;75:93-102.Google Scholar
Liberman  MCChesney  CPKujawa  SG Effects of selective inner hair cell los on DPOAE and CAP in carboplatin-treated chinchillas.  Auditory Neurosci.1997;3:255-268.Google Scholar
Dallos  PWang  CY Biolelectric correlates of kanamycin intoxication.  Audiology.1974;13:277-289.Google Scholar
Slack  RWWright  AMichaels  LFrohlich  SA Inner hair cell loss and intracochlear clot in the preterm infant.  Clin Otolaryngol.1986;11:443-446.Google Scholar
Spoendin  H Retrograde degeneration of the cochlear nerve.  Acta Otolaryngol.1975;79:266-275.Google Scholar
Liberman  MCKiang  NYS Acoustic trauma in cats: cochlear pathology and auditory-nerve activity.  Acta Otolaryngol Suppl.1978;358:1-63.Google Scholar
Herrmann  BSThornton  ARJoseph  JM Automated infant hearing screening using the ABR: development and validation.  Am J Audiol.1995;4:6-14.Google Scholar
Northern  JLEpstein  S Neonatal hearing screening.  In: Lalwani  AK, Grundfast  KM, eds. Pediatric Otology and Neurotology. Philadelphia, Pa: Lippincott-Raven Publishers; 1998:155-162. Google Scholar
Peters  JG An automated infant screener using advanced evoked response technology.  Hear J.1986;39:25-30.Google Scholar
Jacobson  JTJacobson  CASpahr  RC Automated and conventional ABR screening techniques in high-risk infants.  J Am Acad Audiol.1990;1:187-195.Google Scholar
Kileny  PR New insights on infant ABR hearing screening.  Scand Audiol Suppl.1988;30:81-88.Google Scholar
Sinniger  YSHood  LJStarr  ABerlin  CIPicton  TW Hearing loss due to auditory neuropathy.  Audiology Today. March/April 1995:0-13. Google Scholar
Original Article
June 2001

Selective Inner Hair Cell Loss in Premature Infants and Cochlea Pathological Patterns From Neonatal Intensive Care Unit Autopsies

Author Affiliations

From the Departments of Otolaryngology (Drs Amatuzzi, Liberman, and Eavey and Ms Northrop) and Audiology (Drs Thornton, Halpin, and Herrmann), Massachusetts Eye and Ear Infirmary, Boston; Department of Otology and Laryngology, Harvard Medical School, Boston, (Drs Liberman, Thornton, Halpin, Herrmann, and Eavey); Departments of Pediatrics (Drs Pinto and Saenz) and Pathology (Dr Carranza and Ms Northrop), Hospital Nacional de Niños, Escuela Autonomade Ciencias Medicas, Universidad Autonoma de Centro America, San Jose, Costa Rica; and Department of Otorhinolaryngology, Universidade de Sâo Paulo, Sâo Paulo, Brazil (Dr Amatuzzi).

Arch Otolaryngol Head Neck Surg. 2001;127(6):629-636. doi:10.1001/archotol.127.6.629

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.