Early Identification, Early Implantation

Carmen Hayman, AuD, CCC-A, CISC

Morgan, a 7-week-old female, presented to her primary care office with parental concerns due to pregnancy complicated by cytomegalovirus (CMV) infection at 8 weeks gestation and failed newborn hearing screening. Morgan was found to be CMV-positive at birth and was treated with a 6-week course of IV ganciclovir. She failed multiple newborn hearing screenings during her hospitalization. Her PCP recommended audiologic, otologic, and ophthalmologic consultation.

Two days after Morgan’s first primary care well visit, diagnostic auditory brainstem response (ABR) testing at CHOP’s Center for Childhood Communication indicated bilateral sensorineural hearing loss, moderate-to-severe in the right ear and severe-to-profound in the left ear. She was fit with hearing aids by age 10 weeks and enrolled in appropriate early intervention services shortly thereafter.

At age 8 months, behavioral hearing test results indicated hearing loss consistent with previous ABR findings. Morgan experienced persistent middle ear effusion and received pressure equalization tubes at age 12 months. However, parents and early intervention providers expressed concerns about Morgan’s decreasing responses to sound. Subsequent audiologic testing revealed her hearing had progressed to the profound hearing loss range for both ears and she was receiving little benefit from hearing aids. She was referred for a multidisciplinary cochlear implant candidacy evaluation.

Morgan received a cochlear implant in her left ear at age 17 months and in her right ear at 21 months. She enrolled in a preschool program for children with hearing loss that emphasizes listening and spoken language. With her cochlear implants, Morgan has access to speech and environmental sounds in the borderline normal hearing sensitivity range. Her most recent communication evaluation indicates that her speech and language skills are age appropriate.

Discussion

Newborn hearing screening programs identify babies with hearing loss. Early referral to a pediatric audiologist facilitates appropriate hearing technology selection and fitting. The early use of appropriate hearing technology provides the auditory stimulation critical for auditory, speech, and spoken language skill development.

Today, there are many hearing technology options for children, including standard hearing aids, bone-conduction hearing aids, osseointegrated bone-conduction devices, and cochlear implants. For children identified with bilateral severe-to-profound hearing loss or as at high risk for progressive sensorineural hearing loss, early referral to a cochlear implant program is recommended because hearing aids often do not provide access to spoken communication at soft and normal conversational levels.

Evaluation for a cochlear implant can begin as early as 6 months of age with some of the medical evaluations occurring as early as 2 to 4 months. At CHOP, children may receive cochlear implants as early as 9 to 12 months of age, pending insurance authorization. Many children are receiving bilateral cochlear implants. The team explains options for sequential versus simultaneous implantation with the family, and makes recommendations for each individual patient.

Cochlear Implant Candidacy and Management

Early implantation leads to better outcomes for children with bilateral severe-to-profound sensorineural hearing loss. The CHOP Cochlear Implant Program uses a multi-disciplinary approach to evaluation and habilitation.

• Primary Care Physician
  • Refer to audiology for failed newborn hearing screening or cases of progressive hearing loss
  • Monitor and treat otitis media, refer to otolaryngology when indicated
  • Provide additional immunizations when indicated (eg, meningitis vaccinations)
• Otolaryngologist
  • Review MRI of temporal bones to confirm anatomy can support implantation (rule out lesions acoustic nerve lesions, significant cochlear malformation, etc.)
  • Recommend additional evaluations due to bilateral sensorineural hearing loss (e.g., EKG, urinalysis, genetic and ophthalmologic consultations)
  • Perform cochlear implantation procedure and provide post-operative monitoring as needed
• Audiologist
  • Determine severity of hearing loss
  • Confirm limited benefit from hearing aids
  • Recommend sequential vs. simultaneous implantation
  • Work with family to select appropriate external equipment
  • Assess internal device function
  • Program processors
  • Facilitate equipment maintenance
• Speech-Language Pathologist
  • Evaluate speech and language abilities and progress
  • Recommend communication options
• Social Worker
  • Explore expectations and concerns with family
  • Complete developmental milestone questionnaires
• Teacher of Deaf and Hard of Hearing
  • Review IFSP/IEP and make recommendations for changes when needed
  • Liaison between the Early Intervention/Education team and the Cochlear Implant Program

Referral Information

To refer a patient to the Center for Childhood Communication, call 1-800-551-5480.