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Online EHDI Case 6 (Part 2 with Answers)

Online EHDI Case 6: Late-Onset Bilateral Progressive Sensorineural Hearing Loss

Part 2

3 weeks later (Tommy is now 5 months 1 week), you received a phone call from the audiologist who performed the comprehensive evaluation on Tommy. The results indicate a bilateral moderate to severe sensorineural hearing loss (SNHL). The following are the specific findings:

  • OAEs are absent bilaterally
  • Acoustic immittance testing reveal normal middle ear function bilaterally
  • (Tympanometry) with elevated reflex threshold in both ears.
  • Diagnostic ABR reveal responses ranging from 45-80 dB across the frequency range from 500 to 4000 Hz for both ears.

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1. What are some of the reasons Tommy would have passed his newborn hearing screening with this sensorineural hearing loss? 

• This question is likely to come up during your visit with the family. This might also be a good time to acknowledge the parent’s concerns and their correct observation, which lead to this diagnosis.
• Any type of universal screening program needs to achieve a low “false alarm” rate and a high “hit” rate. That is, the goal for UNHS is that as few children as possible are referred for additional, more expensive testing who do not need it (i.e., the test should be specific) and as few children as possible with hearing loss are missed through the screening process (i.e., the test should be sensitive). The sensitivity of current UNHS methods is not 100% and therefore may not identify all children with minimal/ mild hearing losses. Tommy underwent a screening/automated ABR (a-ABR) a few days after birth. An a-ABR is conducted at a low intensity level (35 dB) (REF to Audiogram- their should already be one available from the previous cases) that could at times miss a mild loss (30 dB). In addition, certain medical conditions can exist that can cause a change or progression of hearing loss as well as later onset of hearing loss. That is, normal hearing or a mild undetected hearing loss can be present at birth but may progress to a more severe hearing loss after the child goes home from the hospital. If no further audiological monitoring is being completed within the child’s medical home, the result could be late identification of milder degrees of hearing loss.
• Tommy passed his newborn hearing screen and at this time, the apparent discrepancy between the screen and the diagnostic current test findings is unknown.
     The following PowerPoint presentation discusses 11 cases of late identified children who all passed their UNHS at the hospital. Presented by Wendy D. Hanks, Ph.D. Gallaudet University (PowerPoint)
     The following article also further discusses such discrepancy where 21 infants (30 ears) who failed OAE & passed A-ABR in newborn period had permanent bilateral or unilateral HL at 9 months of age- Johnson, White, Widen, Gravel, James-Trychel, 4 Kennally, Maxon, Spivak, Sullivan-Mahoney, Vohr, Weirather, and Holstrum. "A Multisite Study to Examine the Efficacy of the Otoacoustic Emission/Automated Auditory Brainstem Response Newborn Hearing Screening Protocol: Introduction and Overview of the Study". American Journal of Audiology. Vol. 14 _S178–S185 _ December 2005 http://aja.asha.org/cgi/reprint/14/2/S178.pdf
• Although Tommy does/did not have any medical risk factors (discussed below) which could have warned you (passed newborn hearing screening; no family history; no apparent syndromes), he could have had normal or near normal hearing at birth and his hearing may have changed. It is important that PCPs recognize that not all children who develop progressive hearing loss have risk factors identified at birth. This is why ALL children even if they have passed their newborn hearing screening and have no identified risk factors (see below), need to be monitored with developmental surveillance.

2. According to JCIH 2007, what are some of the risk factors that are listed as greater concern for late onset hearing loss?

RISK FACTORS (JCIH 2007):
• Caregiver concerns regarding hearing, speech, language or developmental delay
• Family history of permanent childhood hearing loss
• In utero infections, such as CMV, herpes, rubella, syphilis and toxoplasmosis
• Syndromes associated with hearing loss or progressive or late-onset hearing loss such as neurofibromatosis, osteopetrosis and usher syndrome
• Neurodegenerative disorders such as Hunter syndrome
• Culture-positive postnatal infections associated with sensorineural hearing loss, including confirmed bacterial and viral meningitis
• Head trauma that requires hospitalization
• Chemotherapy
• The JCIH 2007 Position Statement recommends that children who passed their newborn hearing screening but who also have any of these factors receive at least one audiologic assessment between 24-30 months. However, it is important to remember that progressive hearing loss occurs in some situations in absence of risk factors (e.g., a child with asymptomatic cytomegalovirus). This again is why all children must receive developmental surveillance and why caregiver concerns must be counted as a risk factor prompting audiologic assessment.

3. What are the discussions/recommendations that should be made by the medical home to best serve Tommy and his family?

• First, ensure that the family understands the diagnosis, the implication of this hearing loss and the importance for immediate action in getting Tommy the services he needs. Do they have any questions about the diagnosis itself? The audiologist should have explained it to them; however it is always good to reiterate to ensure that the family is on board. This is a permanent loss that could have a significant impact on the child’s ability to develop speech and language skills and therefore on his quality of life.
• Rapidly progressive hearing loss can be associated with several congenital conditions, including Cytomegalovirus (CMV) and Large Vestibular Aqueduct (a congenital inner ear abnormality on radiographic imaging) as well as some genetically inherited losses.
• Tommy needs to start habilitation right away.
• Referral to early intervention services is essential.
• Non-syndromic hearing loss is when there are no other significant features besides the hearing loss. Syndromic, on the other hand, involves other medical or physical findings in addition to the hearing loss. Therefore, medical workup should include the following disciplines in order to evaluate the possible etiology of the disorder and possible associated medical conditions:
• Pediatric ophthalmology: High coincidence between eye problems and hearing loss such as in Usher Syndrome.
• Pediatric otolaryngology: Tommy should be seen and subsequently followed by a pediatric otolaryngologist who, if at all possible, specializes in seeing children with hearing loss. Continual monitoring of their outer and middle ear status is important to ensure that the presence of excessive cerumen and/or fluid in the middle ear will not add to the sensorineural loss. The
ENT physician would be involved in identifying a possible cause for this hearing impairment and subsequent referral for ancillary testing (CT san / MRI), genetic counseling (see below), and more.
• A genetic evaluation can provide several important pieces of information to the family and the child’s health care providers. A significant portion of cases of early childhood hearing loss are due to genetic causes and mutations. A positive result can alleviate anxiety associated with the worry that other medical problems may arise in their child due to the association of hearing loss with many other syndromes (e.g. Usher syndrome in which blindness can develop at a later age).Other disciplines, which may be consulted simultaneously or later on, are speech pathology, pediatric
cardiology, nephrology, endocrinology and orthopedics.
• Additional resources can be found at
http://www.babyhearing.org/HearingAmplification/Causes/genetics.asp
• Remediation: Tommy needs to be fitted with binaural amplification.
• Continuing audiological evaluation. A second, more detailed ABR needs to be conducted in order to monitor for further change in hearing. This ABR should be repeated before or right around Tommy’s 6 month birthday so that it could be completed without sedation/anesthesia.

 

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