Sound & Silence: A Mother shares the twists and …

1 AllTogetherNow, aboutacollaborationbetweenDMSandDartmout h sThayerSchoolofEngineeringthatisthenatio n smostadvancedefforttodevelopeffectivealt ernativestomammog-raphy( ),and CompoundInterest, abouttwopromisingchemopreventativesthat were developed at Dartmouth and are now in clinical trials( ).At first, after we found out Geneva was deaf, Icouldn t help feeling angry. I d sit there andstare at her perfect-looking little ears as shenursed and think to myself,Howdareyounotwork? But that s when I was still focused on all thatwe had lost or that I thought we would ,thensixweeksold,had,forexample,nev-er heard my hospital where Geneva was born inJanuary 2007, plus a couple of hours of diagnostictests at DHMC in March, to determine that Gene-va was deaf.

2 I m seeing a pattern consistent with profoundsensorineural hearing loss, I remember the audiol-ogist telling my husband and I go to the doctor, I usually can comprehend26 DartmouthMedicine Durgin was born into a totally silent world; she wassix weeks old when she was diagnosed as profoundly Geneva couldn t hear, her parents began learning sign language so they could communicate with her. Here, her Mother signs panda as Geneva looks at a emotion of learningthat your baby wasborn profoundly challenge of learningsign language so you cancommunicate with angst of decidingwhether or not to havea device to help her hearimplanted in her Dartmouth Medicinestaff member sharesthe twists and turnsin an unexpectedparental Jennifer DurginJON GILBERT FOXwhatever medical jargon is thrown my way.

3 Afterall, as a member of this magazine s staff, I read andwrite about medicine every day at my job. But thistime I had to make sense of an unfamiliar termwhile I was trying to contain the emotions wellingup inside me. Soisthatlikewhatyou dcalldeaf? ,Isuddenlywon-dered if the word deaf was politically incorrectand that s why the audiologist had used doctor lin-go. Yes, she answered. I think my next questionwas Are you sure? She was very became clearer for my husband, Chris-tian, and me when the audiologist pulled ,known as an audiogram, had volume (mea-sured in decibels) on one axis and frequency (mea-sured in cycles per second, or hertz) on the otheraxis. It showed the volumes and frequencies foreveryday sounds such as a bird chirping, a dogbarking, a phone ringing, a person talking.

4 It alsoshowed where different speech sounds in the Eng-lish language are found between 20 and 60 deci-bels and 200 and 8,000 hertz. The audiologist ex-plained that she had tested Geneva s hearing up to100 decibels and 4,000 hertz but had seen no brainresponse at all. That meant Geneva could be rightAnAudiogram1252505001,0002,0004,000 8,0000102030405060708090100110120H E A R I N GL E V E LI ND E C I B E L S( d B )F R E Q U E N C YI NC Y C L E SP E RS E C O N D( H z ) DartmouthMedicine29 Fall200828 DartmouthMedicine to a running lawnmower or a helicopter inside I understood that my daughter was deaf. ButIstillhadlotsofquestions. Whatdoessensorineur-al hearing loss mean?

5 I was wondering to are three regions of the ear, the audiolo-gist explained the outer ear, the middle ear, andthe inner ear and different hearing tests can dhad,Geneva souterearand middle ear were just fine. The problem seemedto be in her inner ear, where Sound waves travelLast fall, as Geneva was starting to babble, above, her parents were weighing treatment options,including a cochlear implant. Today, below, with an implant, her hearing is close to the normal range. Loss was the perfect wordfor what I felt over the nextcouple of weeks. She llnever know the Sound of hermother s voice, I remembersaying to my husband onenight, as tears fell frommy eyes onto my the snail-shaped cochlea, and tiny hair cellsconvert those waves into electrical impulses.

6 Theimpulses then travel along the auditory nerve tothe brain. Damaged, dysfunctional, or missing haircells were most likely the cause of Geneva s hear-ing sensorineural partbegantomakesensetome, but I still had trouble with the hearing loss part. How could Geneva, who was only six weeksold, have lost something that she never had?But loss was the perfect word for what I feltover the next couple of weeks. She ll never knowthe Sound of her Mother s voice, I remember say-ing to my husband one night, as tears fell from myeyes onto my pillow. And all those Beatles and BobDylan songs he had been singing her since she wasborn she had never heard a single ,perhapsbecauseI dhadmoreexperiencewithbabiesandyoung children.

7 I could imagine my daughterrunning toward the road and not hearing that ,reaching for something hot, and my not being ableto call out Careful! from across the room. I thinkI also took the news harder because I would bearmore of the load of making sure that Geneva didnotfallbehinddevelopmentally,sinceI dbespend-ing more time with her. I love my job as a writer forDartmouthMedicinemagazine and had when we learned about Geneva s deafness, Iknewsheneededlotsofone-on-oneattentionf romme more than I needed to work and more than myhusband and I needed the money. Happily, howev-er,themagazine seditoragreedtoletmeworkpart-time, so I didn t have to give up working ,wewouldfind ways to lower our feeling a sense of loss over Geneva sdeafnessandmyinabilitytoworkfull-time, ,welearnedaboutallsortsofdis-abilities and syndromes that can accompany ,kid-neyfailure,heartattacks.

8 IfGenevagetsdiagnosedwith one of those syndromes, that s when I ll stopgettingoutofbedinthemorning, I dsay,onlyhalfjoking. My husband and I worried for some timethat there might be another, far worse diagnosislurking in the as the weeks and months passed, and as allthe scary syndromes could finally be ruled outthanks to genetic testing, three EKGs, and an eyeexam at DHMC, Geneva s being deaf felt less andAn audiogram is a pictorial representation of sounds from the rustle of leaves to the roar of a jet engine. When Geneva had her first audiogram, about a monthafter she was born, she showed no response at all to sounds in the area of the red oval; the testing equipment doesn t even produce sounds beyond 100 her most recent audiogram in early May of this year, three months after the activation of her cochlear implant she showed a response to sounds in the areaof the yellow oval.

9 Her hearing would likely be even better now, according to her audiologist, because she has had four more months of experience using the D BNNGE LUZ VFSTHIOARPHGKCHSHJJON GILBERT DartmouthMedicine31 Fall2008able to get one. In the early days of cochlear im-plants, surgeons would often discover such anom-alies in the operating room. Today, sophisticatedimaging techniques give doctors a clear preop lookatachild until late October 2007, when Geneva wasnine months old and her inner ear was more devel-oped, for those tests. And according to FDA guide-lines, she wouldn t be eligible for the surgery untilshe was 12 months old. (Children with less severehearing loss may not be a candidate for an implantuntil they re even older.)

10 In only a very few circum-stances are implants permitted before a child turnsone.) In the meantime, we had a lively, was Tami s job to introduce Christian and meto all the ways of communicating with deaf chil-dren. Some methods depend heavily on any hear-ing a child does have, plus or minus depend on sign language, either AmericanSignLanguage(orASL,anentirelangu agewithitsown grammar), signed English (which uses manyASLsignsbutEnglishgrammarandwordorde r),orcued speech (which uses hand shapes to representphonetic sounds). Given Geneva s degree of hear-ing loss, and the futility of hearing aids, we allagreedthatsomeformofsignlanguagewould makethe most sense at least in the short-term whenGeneva had no access to Sound .