Autologous Bone Marrow Harvest and Transplant for Sensorineural Hearing Loss

Inclusion Criteria:

1. Evidence of sensorineural hearing loss that is,

   • Bilaterally Moderate or Profound in degree
   • Symmetrical or asymmetrical configuration
   • Sudden or progressive in presentation
2. Normally shaped cochlea, as determined by Magnetic Resonance Imaging or computed tomography (CT)

3. The loss must be considered:

   • Acquired
   • Unknown with genetic testing negative. (Genetic testing is not required for Cytomegalovirus (CMV) positive children due to Cytomegalovirus (CMV) known to be number one cause of hearing loss)
4. Fitted for hearing aids no later than six months post detection of loss unless not recommended by treating audiologist or physicians
5. Enrollment in a parent/child intervention program
6. Age 2 years - 6 years old at time of infusion with 2 to 4 years of time elapsed since diagnosis of hearing loss at the time of bone marrow mononuclear fraction (BMMF) infusion.
7. Ability of the child and caregiver to travel to Orlando, and stay for at least 4 days, and to return for all follow-up visits.

Exclusion Criteria:

1. Inability to obtain all pertinent medical records:

   • (pertinent physician notes, speech language pathology notes, laboratory findings, test results and imaging studies-must be sent to the research team at least prior to the subject arriving at the study location for preliminary screening and eligibility assessment, preferably14 days before the scheduled visit.)

2. Known history of:

   • Recently treated (ear or any infections) infection less than 2 weeks before infusion.
   • Renal disease of altered renal function as defined by serum creatinine > 1.5 mg/dl at admission.
   • Hepatic disease or altered liver function as defined by Alanine Transaminase (SGPT) > 150 U/L, and or Total Bilirubin > 1.3 mg/dL
   • Malignancy
   • Immunosuppression as defined by White Blood Cell (WBC) < 3,000 at admission
   • Human Immunodeficiency Virus (HIV)
   • Hepatitis B
   • Hepatitis C
   • Pneumonia, or chronic lung disease requiring oxygen
3. Any evidence of active maternal infection during the pregnancy
4. Participation in a concurrent intervention study
5. Mild hearing loss with no evidence of moderate of severe loss
6. Unwillingness or inability to stay for 4 days following infusion (should problems arise following the infusion) and to return for the one month, six month and one year follow-up visits.
7. Evidence of conductive hearing loss
8. Documented recurrent middle ear infections which are frequent (>5 per year)
9. Otitis media at the time of examination
10. Before 2 years from identification of hearing loss at time of infusion
11. After 4 years from identification of hearing loss at time of infusion

12. Diagnosis of the following syndromic cause for hearing loss

    • CHARGE
    • Waardenburg
    • Brachio-Oto-Renal
    • Pendred
    • Alport
    • Treacher-Collins
    • Usher
    • Stickler Syndrome