OTOLOGIC DIAGNOSIS AND TREATMENT AFTER NEWBORN HEARING SCREENING

ABSTRACT

In the system of hearing screening only high-risk infants, hearing screening is limited to the intensive care nursery. In this system, the high-risk neonatologist is the medical specialist with the responsibility for follow-up examination and diagnosis of young infants with hearing impairment. We are entering an era with the goal of screening all newborn babies for hearing impairment. Instead of examining and testing toddlers aged 1 to 2 years who are newly identified with hearing loss, physicians can expect to diagnose, and sometimes treat, very young infants. This article presents recommendations for the medical examination, diagnosis, and treatment of hearing loss in the growing number of young infants identified with hearing loss.

KEYWORD

Congenital hearing loss - otologic examination - infant hearing screening

REFERENCES

• 1 Busis S N, Busis N A. The neurotologic examination. In: Jackler RK, Brackmann DE, eds. Neurotology St. Louis: CV Mosby 1994: 186-187
• 2 Thelin J W. CHARGE syndrome. Part II. Hearing loss.  Int J Pediatr Otorhinolaryngol . 1986;  12 145
• 3 Bluestone C D, Klein J O. Methods of examination: clinical examination. In: Bluestone CD, Stool SE, Kenna MA, eds. Pediatric Otolaryngology Philadelphia: WB Saunders 1996: 150-164
• 4 Eavey R D. Abnormalities of the neonatal ear: otoscopic observations, histologic observations, and a model for contamination of the middle ear by cellular contents of amniotic fluid.  Laryngoscope . 1993;  103 1-30
• 5 Grundfast K M. Hearing loss. In: Bluestone CD, Stool SE, Kenna MA, eds. Pediatric Otolaryngology Philadelphia: WB Saunders 1996: 249-283
• 6 Fisher N A, Curtin H D. Radiology of congenital hearing loss.  Otolaryngol Clin North Am . 1994;  27 511-531
• 7 Weber P C, Cass S P. Neurotologic manifestations of Chiari 1 malformation.  Otolaryngol Head Neck Surg . 1993;  109 853-860
• 8 Phelps P D, Vezina G. Radiology of the temporal bone: congenital abnormalities. In: Lalwani AK, Grundfast KM, eds. Pediatric Otology and Neurotology St. Louis, MO: Mosby 1998: 181-197
• 9 Reardon W. Genetic deafness.  J Med Genet . 1992;  25 521-526
• 10 Lalwani A, Castelein C M. Cracking the auditory genetic code: nonsyndromic hereditary hearing impairment.  Am J Otol . 1999;  20 115-132
• 11 Tseng C J, Lalwani A K. Syndromic hereditary hearing impairment.  Am J Otol . 2000;  21 437-451
• 12 Estivill X, Fortina P, Surrey S. Connexin-26 mutations in sporadic and inherited sensorineural deafness.  Lancet . 1998;  351 394-398
• 13 Cohn E S, Kelley P M. Clinical phenotype and mutations in Connexin 26 (DFNB1/GJB2), the most common cause of childhood hearing loss.  Am J Med Genet . 1999;  89 130-136
• 14 Starr A, Picton T W, Sininger Y. Auditory neuropathy.  Brain . 1996;  119 741-753
• 15 Rance G, Beer D E, Cone-Wesson B. Clinical findings for a group of infants and young children with auditory neuropathy.  Ear Hear . 1999;  20 238-252
• 16 Doyle K J, Sininger Y, Starr A. Auditory neuropathy in childhood.  Laryngoscope . 1998;  108 1374-1377
• 17 Paradise J L, Bluestone C D. Early treatment of the universal otitis media of infants with cleft palate.  Pediatrics . 1974;  53 48-54
• 18 Jahrsdoerfer R A. External auditory canal atresia. In: Lalwani AK, Grundfast KM, eds. Pediatric Otology and Neurotology St. Louis, MO: CV Mosby 1998: 533-540
• 19 Chang K W, Vohr B R, Norton S J, Lekas M D. External and middle ear status related to evoked otoacoustic emission in neonates.  Arch Otolaryngol Head Neck Surg . 1993;  119 276-282
• 20 Doyle K J, Burggraaff B, Fujikawa S. Neonatal hearing screening with otoscopy, auditory brainstem response and otoacoustic emissions.  Otolaryngol Head Neck Surg . 1997;  116 597-603
• 21 Doyle K J, Rodgers P, Fujikawa S, Newman E. External and middle ear effects on newborn hearing screening tests.  Otolaryngol Head Neck Surg . 2000;  122 477-481
• 22 Jaffe B F, Hurtado F, Hurtado E. Tympanic membrane mobility in the newborn (with seven months' follow-up).  Laryngoscope . 1970;  30 36-48
• 23 Balkany T J, Berman S A, Simmons M A, Jafek B W. Middle ear effusions in neonates.  Laryngoscope . 1978;  88 398-405
• 24 Klein J O. Risk factors for otitis media in children. In: Kavanagh JH, eds. Otitis Media and Child Development Parkton, MD: York Press 1986: 45-51
• 25 Marchant C D, Shurin P A, Turczyk V A. Course and outcome of otitis media in early infancy: a prospective study.  J Pediatr . 1984;  104 826-831
• 26 Weber P C, Perez B A, Bluestone C D. Congenital perilymphatic fistula and associated middle ear abnormalities.  Laryngoscope . 1993;  103 160-164
• 27 Reilly J S, Weber P C. Perilymphatic fistulas in infants and children. In: Bluestone CD, Stool SE, Kenna MA, eds. Pediatric Otolaryngology Philadelphia: WB Saunders 1998: 371-377
• 28 Wazen J J, Caruso M, Tjellstrom A. Long-term results with the titanium bone-anchored hearing aid: the U.S. experience.  Am J Otol . 1998;  19 737-741
• 29 Fortnum H M. Hearing impairment after bacterial meningitis: a review.  Arch Dis Child . 1992;  67 1128-1133
• 30 Nadol J B. Hearing loss as a sequela of meningitis.  Laryngoscope . 1978;  88 739-755
• 31 Odio C M, Faingezicht I, Paris M. The beneficial effects of early dexamethasone administration in infants and children with bacterial meningitis.  N Engl J Med . 1991;  324 1525-1531
• 32 Novak M A, Fifer R C, Barkmeier J C, Firszt J B. Labyrinthine ossification after meningitis: its implications for cochlear implantation.  Otolaryngol Head Neck Surg . 1990;  103 351-356