Key note: Bilateral restoration of hearing A. Aschendorff, T. Wesarg, S. Kröger, R. Beck, R. Laszig, S. Arndt Dept. of Otorhinolaryngolgoy and IMplant Center Freiburg University of Freiburg

Implant Center Freiburg (ICF) CI, ABI, implantable hearing devices Re-habilitation and long-term-care > 2500 CI patients > 250 CI surgeries /y. Adults + children Center of competence Baden-Württemberg Interdisciplinary team: ENT, audiology, speech and language therapy, linguistics, psychology, music therapy, technicians… (n=44) certified rehabilitation center (BAR)

Age at surgery NHS, diagnostics and HA experience for 4 m., decision at 8 m. Surgical point of view: ideal at/around 10 m. Surgical technique: atraumatic Specialized centers for surgery in the very young? Data on even earlier surgery? (Colletti et al. 2012…

Fact and fiction? – Age at surgery (Germany) 2012 % Significant increase for surgeries <24 m. to 2006: 25,2% <2 y., 2006-2012 46,3% < 2 y. still, 53,7% of children are older than 24 m. Reason? Late provision with HA, reservations against early surgery, add. med. factors, progressive hearing loss! (Fitzpatrick et al. 2011)

Long term outcome vs. age at surgery Monosyllables (> 5 y Long term outcome vs. age at surgery Monosyllables (> 5 y. post CI) Laszig et al. 2010, Dissertation R. Beck, Richter et al. 2002, Tajudeen et al. 2010, de Raeeve 2010, Colletti et al. 2011 *** m 1,3 J. n=38 m 2,7 J. n=139 m 7,2 J. n= 86 m 45,4 J. n=199 Group A with superior resuts Group D: adults, congenital as well as progress. HL

Bilateral CI German "Guideline CI": bilateral CI whenever possible Schafer et al. 2011 (Int J Audiol) "A meta-analysis to compare speech recognition in noise with bilateral cochlear implants and bimodal stimulation" (42 studies analysed): binaural advantages in bilaterale CI`s: squelch (central noise reduction), summation, head-shadow effect advantage compared to bimodal stimulation German "Guideline CI": bilateral CI whenever possible German health insurance: bilateral CI in cases at risk of or with obliteration/meningitis all others: single-case decision, sequentially or simultaneously All centers: increasing number of bilaterals, no combined approach UK: National Paediatric Bilateral Cochlear Implant Audit

Bilateral situation in Freiburg N= 2234 (Stand 09-2012) Mean intervall 1st to 2nd implant (all p. up to 18 y.): 5.9 years! Since 2006: significant reduction to 1.7 y. improved counseling Aim: 2nd CI within 12 m. Sequentiell Kinder n=229 Kinder n=281 Intervall in Jahren, Alter bis 12 J. Bilaterale n=446

Late sequential BCI: Results OLSA at 70 dB in quiet, Late sequential BCI: Results OLSA at 70 dB in quiet, *progressive HL, n=15, mean interval between 1st and 2nd CI: 8 y., (r: 4-12 y.) Delay: necessary time to reach results of 1st ear or stable longterm result Not all will acchieve identical results, chance of drop out and, finally, rejection of 2nd implant *develop *develop *numbers Delay: <1y. 1y. 2y. 3y. 4y.

The sequential problem Bilateral advantage correlated to time between surgeries, interval >8 y. seems critical Steffens et al. 2008 (H, RB, FR) Late sequential BCI: negative effect visible at time interval of >12 M, Gordon et al. 2008 Exemption: progressive hearing loss!

…and… where´s the beef? New field: auditory rehabilitation in single-sided deafness and asymmetric hearing loss Similar considerations, similar factors to influence results

Why bother? 70-93% report difficulties for speech comprehension in noise, independent of age (Coletti et al. 1988; Ruscetta et al. 2005; Priwin et al. 2007; Wie et al. 2009) 12-41% of children require additional support in school (Bess & Tharpe 1986; Bovo et al. 1988) 22-35% of children have to repeat classes (Bess and Tharpe 1986; Brockhauser et al. 1991; Cho Lieu et al. 2004) Misinterpretation hyperactivity Results in adults are encouraging therapy in children as well

Incidence of unilateral HL in NHS Unclear! Berninger & Westling 2011: NHS (6 y., >30.000) bilateral HL 0.17%, unilateral 0.06% (Ratio 3:1) Ghirri et al. 2011: NHS, (>7000), unilat. HL 0.99/1000 Nie 2008, (China): bilateral HL 2.22/1000, unilateral 2.74/1000 newborn

Etiology adults (n=101) n=93 postlingual, n=10 congenital, n=10 AHL

Freiburg: only n=8 qualified for a CI! Etiology children (n=16) n=11 congenital, n=4 erworben, n=1 non organic Etiology (incl. 1 CHARGE) Kutz et al. 2011: deficient nerve: limited results, absent nerve: poor results Freiburg: only n=8 qualified for a CI!

Eiology children SSD I Unilateral LAV in n=2 children

Etiology children SSD II HRCT: IAM normal, MRI: CND/aplasia of VIIIth nerve, n=5 CHARGE, N VII aplasia R, N VIII aplasia L

SSD: treatment options? no therapy conventional CROS-HA BAHS for CROS Vanecloo et al. 2002 Cochlear Implantation Vermeire et al. 2008; Arndt et al. 2011 Adults! 17

Rehabilitation in patients with unilateral deafness Study design primary objective: audiometric test results (speech discrimination in noise, localisation) unaided, Baha, CROS-HA trial period for each device 3 weeks (Baha BP100 headband/CROS-HA; Phonak Una M) CI recommended, if patients fitted the inclusion criteria (duration of deafness ≤ 10 years, intact auditory nerve) secondary objective: results after 12 months with chosen device (CROS; Baha; CI) comparison between the devices subjective evaluation with SSQ scale

Rehabilitation in patients with unilateral deafness Audiometric test test setup speech test HSM-sentences in noise (Hochmair-Desoyer et al. 1997) sentences @ 65 dB SPL, noise @ 65 dB SPL, SNR 0 dB  % correct answers

Rehabilitation in patients with unilateral deafness Localisation test test setup stimuli OlSa sentences (Wagener et al. 1999) mean sound level: 65 dB SPL level randomisation: ± 6 dB SPL  localisation error [°]

Rehabilitation in patients with unilateral deafness Patients overall: 101 adult patients mean duration of deafness: 10.31 years (1 month - 47.6 years) decision CROS Baha CI No. of patients after test trial (outside CI inclusion criteria) 12 (8) 19 (12) 45 No. of patients with 12 month results (outside CI inclusion criteria) 7 (3) 16 25 drop out 8 all devices rejected not yet decided 5 ~ 25 %

Speech discrimination and localisation results: CROS group (n=7) * * * correct [%] error [°]

Speech discrimination and localisation results: Baha group (n=16) ** * # ** * * * ** # error [°] correct [%]

Speech discrimination and localisation results: CI group (n=25) *** ** ** *** *** # * *** *** ** *** * *** ** ** * *** * correct [%] error [°]

Comparison of speech discrimination and localisation: Results after 12-month device use # * error [°] correct [%]

Comparison of speech discrimination and localisation: Results after 12-month device use # *** *** * # # *** * * correct [%] error [°]

Correlation of benefit (aided - unaided speech understanding) in noise in relation vs. duration of deafness CROS Baha CI benefit/ % duration of deafness/ years

Correlation of speech understanding in SciNnh vs Correlation of speech understanding in SciNnh vs. duration of deafness after 12 months CI use r=-0,616649 (p=0,001, **) SV / % = -5,336999 * duration of deafness / years + 63,667 otosclerosis

Correlation of benefit (binaural/12 months CI use) - monaural /preop) to duration of deafness r=-0,589063 (p=0,001947, **) SV Benefit / % = -4,679889 * duration of deafness / years + 52,505605 labyrinthitis

Subjective evaluation after 12-month device use - SSQ scale *** *** *** ** * * * * speech spatial quality

Conclusion after 12 months device experience: superior results with CI in speech discrimination in all conditions significant improvement in localisation with CI outcome/ rehabilitation time may be correlated to duration of deafness, training necessary patients with long duration of deafness rather benefit from Baha or CROS device CI with long duration of deafness: more data necessary  critical question: duration of deafness to still recommend CI If patients are within inclusion criteria for CI: first choice  CI second choice  Baha or CROS (better results with Baha) Baha and CROS cannot prevent deprivation of the auditory pathway

…and in children? Speech in quiet (n=3) Freiburg monosyllables @ 70 dB child 3 (6 y., congenital, Göttinger I, per audio input , 70 dB): 3 m.: 40% monosyllables 6 m.: 50 % monosyllables

SSD children vs. adults: Results after 12 m., acquired deafness Unknown: development in congential deafness? Sensitive phases? Maximum duration of deafness? Kind 1 Kind 2 Erwachsene (n=22) CI *** unaided with CI OLSA @ 65 dB, Pegelrandom. stimuli noise CI Sprache Lärm child 1 child 2 adults (n=22) ** HSM @ 65 dB, S/N 0 33

Bilateral restoration of hearing In bilateral HL: bilateral CI or bimodal stimulation Results: UK BCI Audit New: auditory rehabilitation in SSD and AHL Evaluation similar to regular CI, MRI necessary Pseudostereophonic BAHS, CROS, Bonebridge with poorer results, but may be indicated in special cases Age, duration of deafness, prior surgery like AN, aplasia of VIIIth nerve, ossification… Training necessary Children: congenital SSD: early treatment, results? acquired SSD: results comparable to adults

Unaided speech understanding in noise in relation to duration of deafness CROS Baha CI % correct duration of deafness/ years