Notes from an Otolaryngologist

By Brian Herrmann, MD, Children’s Hospital Colorado 

Pursuing my career as a Pediatric Otolaryngologist in Atlanta and Denver over the past 15 years has taught me that practicing medicine is (and should be) a partnership with families, using medicine to help children be on a path to a healthy and successful life. While I love the science of medicine, it is the relationships with the children, their families, and my colleagues that I find most meaningful.

Experiencing my own hearing loss worsen as an adult has also given me perspective on how much this can affect daily life. From these experiences, I have developed a set of guidelines that I follow when treating children who are deaf/hard of hearing, highlighting common questions I am asked when discussing care options.

Despite these treatment choices being very different from one another, both families chose the best option for themselves, and I fully supported both decisions. Every treatment decision is individualized, and it hinges upon informed parental choice.

Respectful Team Advocacy

My job as a physician is to answer as many questions as possible. Providing information is one of my key duties, and answering “why?” is as important as “what do we do now?”  These discussions range from hearing physiology to current treatment options, including sign language, amplification, and/or cochlear implantation. Central to this discussion is my attempt to provide unbiased information for the family to build a foundation from which to make sound decisions for themselves. As an example, five years ago I saw two consecutive families with newborns identified with severe to profound hearing loss. After a long discussion of treatment options, the first family chose a full medical work-up and to pursue a listening-speaking approach. The second child was born to parents in the Deaf community and chose a sign language approach. Despite these treatment choices being very different from one another, both families chose the best option for themselves, and I fully supported both decisions. Every treatment decision is individualized, and it hinges uponinformed parental choice.

Caring for children with hearing loss is a long-term commitment, building a strong treatment relationship between family and healthcare providers over years. The advantages of having a dedicated team to care for these children and support the entire family cannot be overstated. A diverse team ensures that all different aspects of hearing care are addressed. Advocacy can take many forms, ranging from determining the best treatment plan to interactions with primary providers, teachers, and caregivers regarding language, scholastic, and social success. Even with minimal interventions, continued success requires careful follow up care and monitoring. I also frequently tell families who are considering cochlear implantation that placing the implant is the start of a journey, not its completion. Audiologists and speech therapists are often more important than the surgeon to CI success.

Evaluation Options

In the course of my discussions with families, the question “how did this happen?” inevitably arises. As a medical professional, I also want to find out the underlying cause to ensure the correct treatment options are provided to the family. Giving a plan for evaluation and the rationale behind these recommendations are equally important parts of this discussion.

Frequently, the next question asked is “Is this reversible?”  The answer to this often resides in the underlying cause, and that is why a thorough evaluation is important. While conductive hearing loss arising from the middle ear (tympanic membrane and ossicles) may be reversible, the location and level of loss, as well as age of the child play into the treatment decisions. For sensorineural hearing loss arising from the inner ear, this is usually permanent. Auditory neuropathy is less straightforward, as this form of hearing loss is not volume loss as much as it is distortion. While some forms of auditory neuropathy are reversible, most are not.

My current evaluation recommendations include:

• Genetic evaluation:
• Studies have suggested genetic mutations as the most common source (60-70%) for bilateral sensorineural hearing loss and auditory neuropathy. 80% do not have a family history for hearing loss.
• Ophthalmological (eye) evaluation:
• Studies suggest up to 25% of children with hearing loss will need glasses or will have something identified in the eye (retina, lens, cornea) that correlates with the diagnosis for the hearing loss.
• Neuroimaging of the ear and brain to evaluate any structural issues in the ear (malformations)
  •  For conductive hearing loss, a CT scan is typically chosen.
  •  For sensorineural hearing loss, an MRI provides better images.
  • CMV (cytomegalovirus) testing has recently garnered more attention from the medical community. Improved testing has identified CMV in a much higher number of children with hearing loss previously attributed to an unknown cause. Early identification and treatment of congenital CMV for those babies referred from newborn hearing screening has demonstrated hearing improvement in some small studies. CMV is an area of active investigation.

Treatment Options

When discussing treatment options with families, I employ two important principles: advocating aggressive early intervention and support, and reserving surgery as the option of last resort. Studies have shown that early and consistent intervention for hearing loss leads to better outcomes. Early comprehensive care is also better than limited interventions. Early use of amplification and speech therapy are advocated whenever possible. Surgery to address ossicular (middle ear) sources for loss can be very successful, the timing can vary immensely depending on the child’s age and underlying source for the conductive loss. I often speak in terms of risk/benefit ratio for this option. Is the level of loss enough to warrant surgery?  No one wants to have a perfect surgical result that fails to improve hearing. For many forms of conductive and sensorineural hearing loss, amplification is the best option.

There continue to be controversies surrounding cochlear implantation. For every person skeptical about CI, there is another calling for earlier and more liberal use of the technology. So many have opinions, and it is sometimes difficult to know what to believe. Because everyone’s personal experiences and opinions regarding deafness is different, it would be unfair to judge one as any more valid than another. That being said, I firmly believe opinions should never restrict families from exploring all of their options.

When determining whether a child is an appropriate candidate for cochlear implantation, I am often asked whether one or both ears should be considered in children with bilateral severe/profound loss. When I began my practice, second side cochlear implantation was considered “experimental”. As time passed, outcome studies demonstrated clear benefits of binaural CI use. A natural extension of this approach led to bilateral simultaneous CI placement. Although these decisions are individualized after a thorough discussion with the family regarding all options, I now offer this to all children with congenital or postmeningitic bilateral severe/profound sensorineural hearing loss and selectively with auditory neuropathy. As perspectives of both families and the medical community have changed, bilateral CI and now bilateral simultaneous CI placement have become the standard of care for appropriate candidates.

Future Considerations

The evaluation and treatment of hearing loss has undergone tremendous growth over the last 20 years. Science has become quite sophisticated in understanding why hearing loss occurs, as well as identifying the nuances within each child’s environment that may influence treatment success.

Taking full advantage of this continuous cycle of improvement requires a treatment team dedicated to offering the latest technology, medical and surgical techniques, and comprehensive care to the family. Collaboration with other research partners across the world ensures the latest advancements are incorporated into an individualized treatment plan for every child our team at Children’s Hospital Colorado sees. While I am confident that continued innovation in hearing aid and cochlear implant technology will provide further advancements in sound awareness, I am also on the lookout for future treatments (like hair cell regeneration) that may eventually supplant CI as the treatment of choice. This is an exciting time to be involved in the care of children who are deaf/hard of hearing, and I am thankful to work with each unique family who comes to our clinics.   ~

H&V Communicator – Summer 2019