TUMOR AFFECTING HEARING

Trials and Tribulations

ABIGAIL KOEHLER, BS
ROHAN RAO, BS
EASHIKA CHAKRABORTY
ABDELKADER MAHAMMEDI, MD
RAVI N. SAMY, MD, FACS
SOMA SENGUPTA, MD, PHD, FRCP

Within the brain, there exist a collection of nerves that function to control the sensation and motor functions of the head. One of these is called the vestibulocochlear nerve or cranial nerve VIII (CN VIII), which receives balance and hearing information from the structures of the inner ear.¹ As with most nerves throughout the body, CN VIII is surrounded by a coating of fat, termed myelin, speeding its conduction of electrical signals. The myelin sheath has a role similar to the insulation surrounding typical copper wiring. Both myelin and this insulation serve to speed up the electrical conduction along the length of the neuron and copper wire, respectively. Within the nervous system, two cell types, oligodendrocytes and Schwann cells, function to produce this myelin and properly envelope the axons. Oligodendrocytes are primarily found within the head and spinal cord whereas Schwann cells are found in peripheral nerves which connect the head to the rest of the body.² As can be insinuated from the name of the disease, a vestibular schwannoma involves a growth of the Schwann cells involved in creating the myelin around CN VIII.

Vestibular schwannomas (VS), also known by the term acoustic neuroma, have an incidence of approximately 3 cases per 100,000 patients/year.^3,4 The characteristic symptoms of VS include loss of hearing, imbalance, and ringing in the affected ear.⁵ These symptoms are caused by a compression of CN VIII due to the expanding VS. As the tumor progresses, it may impinge on surrounding neuroanatomical structures next to CN VIII. One such structure is the cerebellum which is next to CN VIII’s connection in the brainstem. Consequently, late-stage VS can manifest with limb dyscoordination, termed ataxia, and a diminishment of hand-eye coordination, termed dysmetria. Like many other disease processes, VS can be caused by genetic disorders, specifically the neurofibromatosis type 2 (NF2) gene. NF2 is relatively rare and constitutes only 5% of all VS.⁶ Bilateral VS is the defining characteristic of NF2.⁷ Below is the story of Jess, a small-town sock-lover who had to travel across the United States to receive care for her aggressive, fast-growing vestibular schwannoma.

At the young age of 26, Jess learned what it was really like to be an advocate. Beginning as early as 2009, Jess noticed she began having headaches that felt like Charley horses in her head. Her hearing also seemed to become impaired, and everything sounded as if she were underwater. She scheduled an appointment to see her primary care physician, who suggested she had a sinus infection with fluid in her ears. She was prescribed a common antibiotic and sent home to rest and recover. The only issue was, after completing her antibiotic regimen, Jess still had symptoms. After visiting with her doctor again, she was referred to a dentist with the potential diagnosis of grinding her teeth in her sleep. However, her dentist did not see evidence of this. After weeks of pain and no answer, Jess suggested to her doctor that she believed she should get a magnetic resonance image (MRI). Jess underwent imaging with a sneaking suspicion that something was wrong. Her fears were confirmed when her doctor called her on the phone and asked her and her husband to stop by the office. Her doctor was in more shock than Jess as she broke the news that Jess had a brain tumor. (Fig. 8.1). Jess’s gut instinct was correct; there was something else going on in her brain.

Her tumor was a result of a genetic disorder Jess never knew she had called neurofibromatosis type 2 (NF2). She was diagnosed with a vestibular schwannoma tumor on the left side of her brain, which was pushing on her brainstem and wound around bundles of nerves. Later on, her mom found out that Jess’s biological father was diagnosed with NF2 with similar symptoms to Jess and later passed away from his condition. Jess’s siblings were tested but none have the condition.

Jess’s physician referred her to many different Kentucky doctors who would fall short of being her advocate, “I’m sorry Jess, but this tumor is too complex, too big.” Jess found herself terrified for her future as five different doctors could not seem to help her. To her, it seemed as though it were time to go home and die. She thought about how complicated her situation must be if so many people who train for this kind of work were not able to help.

One major advocate in her corner pulled through for her. Jess’s mom was unaccepting of the fact that no one would help her daughter. She contacted the House Ear Institute in Los Angeles, CA to recruit Dr. Derald Brackmann to perform her difficult surgery. He planned to additionally place an auditory brainstem implant (ABI). ABIs are surgically placed at the brainstem of a deaf patient and use an electrode to bypass damaged areas of the brain and create signals for the auditory nerve directly. Jess continued to work her job at a local bank up until the day she flew out to California. She felt very nervous but knew Dr. Brackmann was her only hope at combating her new diagnosis. Jess recalls the most difficult arrangement of her trip was figuring out who could take care of her dog! She expected to stay in California for 2-3 months, with 3-4 of the weeks in intensive care. Her entire surgery lasted 16-18 hours, but she was shocked when she woke and realized she only needed to spend about five hours in the intensive care unit (ICU). Unfortunately, she was unable to get the ABI due to her insurance denying her claim at the last minute. She stayed in LA for two weeks, staying in the hospital for only three days. She she did not need rehab or any other post-operative care. Jess remembers recognizing she was deaf in her left ear, but decided it was okay because she was alive.

Since there were not any physicians in Kentucky who could offer her treatment, Jess applied to the National Institute of Health (NIH) for a study. Jess thinks it is the craziest thing she has ever done in her life. She would hop on a plane to Washington, D.C. to receive chemotherapy, and fly back home. It was around this time that the tumor on her right side began to grow, and her hearing declined. She sought out Dr. Trent Hummel at Cincinnati Children’s Hospital Medical Center, an expert in the field of central nervous system (CNS) tumors.

One day, however, Jess woke up completely deaf. Her hearing loss was later confirmed through the hearing tests she received regularly for following an Avastin chemotherapy protocol. Upon losing her hearing, she also lost her job. She was in touch with Dr. Ravi Samy, a neurotologist/skull base surgeon, at the University of Cincinnati Medical Center, for possible surgical treatment of her NF2. They discussed surgical removal of her tumors, radiation therapy, cochlear implantation, and placement of an ABI (Fig.8.1). Since Jess and her husband were unable to have biological children, they chose to adopt. She and her husband adopted two children who would play a significant role in Jess’ treatment decisions. Although it was an uncertain time for her, she relied on her faith and family and ultimately decided that, despite the risks, “This may be a great opportunity to hear my kids for the first time.” Subsequently, she underwent right-sided radio-surgery, a focused beam of radiation therapy, with her radiation oncologist, Dr. Luke Pater. Six weeks later, following the completion of her radiation therapy, she underwent cochlear implantation.

Before her implant, Jess read lips and communicated in sign language but found many barriers with this. When her cochlear implant was finally turned on, she heard her kids for the very first time. Later on, Dr. Samy informed Jess that she was still a candidate for the ABI during a follow-up appointment. He and Dr. Mario Zuccarello performed her ABI surgery at the University of Cincinnati and within six months she went from deafness to surround sound! Jess exceeded expectations with a 93% response to the ABI and, again, recovered quickly with no complications.

Throughout her journey, Jess has focused her attitude around two things: faith and crazy socks. Jess believes her faith carried her through her difficult journey and gave her a positive outlook on life. As for her love for crazy socks, Jess feels you cannot have a bad day if your feet are having a party! She claims she does not own any plain socks and her care team wears them during her treatment visits, follow-up appointments, and even her surgeries.

Jess’s family is very supportive of her. Her husband was initially very worried about her condition and struggled not having answers or the ability to fix it. Her children grew up knowing about their mom’s condition and understanding she was unable to hear them. Jess says that from the beginning she and her husband were very open with them about her condition. She brought them to doctor appointments, and they would sit with her while she received chemotherapy or watch through the window as she underwent radiation therapy. Jess loves to read and spend time with her kids as a stay-at-home mom. She volunteers at her children’s school letting kids read to her. She also participates in Disability Day at their school by creating a mini station on the topic of deafness to educate children. She spends so much of her time advocating and educating that her young niece views her as a role model and sometimes wears a Cheeto behind her ear because it looks like Jess’s hearing aid (Fig. 8.2).

Even through the most difficult times in her life, Jess has remained optimistic and thankful. Her advice to others who may be experiencing something like her situation would be to not stop just because someone else says you cannot do it. She reflects on her own self-advocacy, because if she had not asked for the initial MRI, her outcome may have been different. She also recommends keeping a positive attitude, saying “Life is so much better if you start looking for the good things instead of what could go wrong; think about what could go right. It’s not ‘I have to have,’ it’s ‘I get to have.’” Jess personally only allows herself one day to cry and be upset, and then she reframes her attitude to match the motto she created for herself. Recently, Jess has scored a 98% on her follow-up hearing test, which is better than most people score. For Jess, life sounds good!