Southern New Hampshire University Cochlear Implantation Hearing Discussion Identify an article that discusses and provides support for a therapy, intervent

Southern New Hampshire University Cochlear Implantation Hearing Discussion Identify an article that discusses and provides support for a therapy, intervention or supportive device that assists individuals with vision or hearing impairments. You will then provide a review of the article including a brief description of the intervention, the evidence supporting the use of the intervention and how it benefits this population. The document should be no longer than 2 pages. The assignment must include references in APA format. Eur Arch Otorhinolaryngol (2015) 272:3201–3207
DOI 10.1007/s00405-014-3376-9
OTOLOGY
Cochlear implantation in patients with Cogan syndrome:
long-term results
Andrea Bacciu • Enrico Pasanisi • Filippo Di Lella
Maurizio Guida • Salvatore Bacciu •
Vincenzo Vincenti

Received: 18 July 2014 / Accepted: 25 October 2014 / Published online: 4 November 2014
Ó Springer-Verlag Berlin Heidelberg 2014
Abstract The objective of this study was to evaluate the
long-term outcomes of patients with Cogan syndrome (CS)
who have undergone cochlear implantation. Subjects consisted of 12 cochlear implant users with a typical form of
CS. Measures included word and sentence recognition
scores. The speech recognition performance was rated
before cochlear implantation and at 1 and 5 years after
implantation. The speech materials were presented in quiet
only condition. The mean 12-month post-operative word
and sentence recognition scores were 91.4 and 93.1 %,
respectively. Five years after implantation, the group
means for word and sentence recognition tests were 94 and
96.3 %, respectively. No patients in this series experienced
flap complication or other local or systemic complications.
This long-term study on 12 subjects with CS over 5 years
of cochlear implant use reveals that cochlear implantation
is safe in the long term and provides excellent and stable
hearing results.
Keywords Cochlear implant Cogan syndrome
Deafness Ossified cochlea Results
Introduction
Cogan Syndrome (CS) is a rare autoimmune vasculitis
characterized by recurrent ocular inflammation associated
with vestibulo auditory dysfunction [1, 2]. Cogan
A. Bacciu (&) E. Pasanisi F. Di Lella M. Guida
S. Bacciu V. Vincenti
Department of Clinical and Experimental Medicine, Unit of
Audiology and Pediatric Otorhinolaryngology, UniversityHospital of Parma, Via Gramsci 14, 43123 Parma, Italy
e-mail: andreabacciu@yahoo.it
syndrome has been divided into classic and atypical forms
on the basis of the ocular findings. The classic form, first
described by Dr. David G. Cogan in 1945 [3], is characterized by the presence of bilateral non-syphilitic interstitial keratitis and vestibulo auditory dysfunction which is
usually bilateral and mimic the more common Ménière
disease with tinnitus, vertigo and sensorineural hearing loss
(SNHL) [4, 5]. The audiovestibular manifestations may
develop coincidentally or within 1–2 years after the onset
of ocular signs [6, 7]. The atypical form may involve every
ocular structure, leading to conjunctivitis, scleritis, uveitis,
retinitis, and optic neuritis. In atypical CS, audiovestibular
symptoms are different from Meniere-like episodes and
appear more than 2 years after ocular manifestations [2].
Systemic disease arises in about 70 % of cases and
includes fever, headache, myalgia, arthralgia, lymphadenopathy, hepatosplenomegaly, aortitis, coronary arteritis,
pulmonary nodules and pleuritis [4, 5]. Systemic vasculitis
develops in up to 10 % of patients and may involve any
sized vessels [4, 8].
The ocular inflammation generally responds to topical
atropine and ocular corticosteroids [9, 10]. Audiovestibular
dysfunction requires systemic corticosteroids and, in
selected cases, immunosuppressive drugs such as methotrexate and cyclophosphamide [1, 10, 11]. Despite this
aggressive treatment, approximately 50–85 % of patients
with CS experience severe to profound irreversible SNHL
[4, 7, 12, 13]. In deafened patients in whom no benefits are
obtained from conventional hearing aids, cochlear
implantation is an highly effective technique in restoring
hearing abilities.
Due to the rarity of the disease, few reports have specifically studied the use of cochlear implants in patients
suffering from CS [4, 5, 8, 10, 14–18]. These studies have
already demonstrated the efficacy of cochlear implantation
123
3202
in patients with CS. However, the majority of the articles
report small series or case reports with a limited follow-up.
This study aimed at evaluating the long-term outcome
performance in a series of 12 patients with CS who
received a cochlear implant. To the best of our knowledge,
this is the largest series of cochlear implantation in patients
with Cogan syndrome.
Materials and methods
After institutional review board approval, a retrospective
analysis of all patients entering the cochlear implant
program at the Cochlear Implant Center of the UniversityHospital of Parma was conducted to identify the patients
who were diagnosed pre-operatively with CS. Twelve
patients with a confirmed diagnosis of CS who received
cochlear implants were identified and participated in the
study. This patient population accounted for 4.4 % of all
implanted patients in our center. This high percentage may
be explained by the presence in our Hospital of a reference Center for the treatment of autoimmune diseases. All
the 12 patients were affected by the classic form of CS.
The diagnosis of the classic form of CS was made on the
basis of the observation of recurrent interstitial keratitis,
acute-onset SNHL, Ménière’s-like audiovestibular dysfunction, and after exclusion of all other causes of interstitial keratitis (i.e., syphilis, sarcoidosis, leprosy, Lyme
disease, viruses, hypersensitivity to drugs). Fluorescent
treponemal antibody absorption test and tuberculosis skin
test were performed in all patients. Laboratory and
immunological tests completed the diagnostic work-up.
One month before surgery, all patients were pre-operatively investigated with high-resolution computed tomography (HRCT) of the petrous bones and magnetic
resonance imaging (MRI) scans of the brain focusing on
the inner ear.
Data collected from the patients’ medical records
included demographic information, operative procedures,
radiological features, intra- and post-operative complications, and performance outcomes.
Speech perception was evaluated using word and
everyday sentence speech recognition tests. Italian versions
of the North-western University Phonetically Balanced
Word Lists and the Central Institute for the Deaf Everyday
Sentence Lists were used to measure speech perception
benefits. The speech materials were presented in hearingonly conditions using a monitored live voice through the
sound field at a level of 70 dB sound pressure level. For the
present study, results were collected for three specific time
intervals: before surgery, at 12 months post-implantation,
and after a minimum of 5 years of follow-up. Follow-up
was defined as the period of time from surgery to the most
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Eur Arch Otorhinolaryngol (2015) 272:3201–3207
recent office visit. The follow-up of the series ranged from
64 to 158 months (mean, 94.7 ± 29.3 months).
Results
A summary of the characteristics of the 12 subjects
included in this study is shown in Table 1. Five patients
reported previously are included in this study with longer
follow-up [18]. There were 8 (66.6 %) women and 4
(33.4 %) men, providing a female–male ratio of 2:1. Preoperatively, all patients exhibited either complete deafness
or a bilateral profound SNHL (unaided pure tone average
thresholds greater than 90 dB). All patients had been fitted
with hearing aids immediately after diagnosis of hearing
loss. In all cases cochlear implantation was considered
when any progress in speech perception abilities from
amplification and rehabilitation was noted. Age at
implantation ranged from 16 to 52 years (mean
34.1 ± 10 years). The length of the time from initial
deafness diagnosis and cochlear implantation ranged from
6 to 48 months (mean 19 ± 13 months). All patients but
one received pre-operative steroid and immunosuppressive
therapy.
Four patients received the Nucleus 24M device
(Cochlear Ltd., Melbourne, Australia); one, the Nucleus
22M device; and two, the Nucleus Contour model; and five
patients were implanted with the MXM Digisonic device
(MXM Corporation, Antibes, France). On pre-operative
HRCT scan, 10 subjects (83.3 %) had normal cochleas and
2 (16.7 %) had some degree of ossification of the inferior
segment of the basal turn of the cochlea; MRI confirmed
the two cases of ossification detected by HRCT and
revealed another case of ossification. At surgery, the three
cases with abnormal radiologic findings were proven to
have cochlear ossification; cochlear ossification was an
intraoperative surprise in other three cases. In total, six
patients (50 %) were found to have some degree of ossification. In four cases, there was bone obliteration confined
to the round window niche and the first millimeters
(2–3 mm) of the inferior segment of the basal turn; however, these were successfully managed by tunneling
through the ossified portion of the basal turn. In the other
two cases, the inferior segment of the basal turn was
completely filled by fibrous and bony tissue, and the
electrode was inserted into the scala vestibuli. We did not
have cases of ossification of the ascending segment of the
scala tympani or of the scala vestibuli. In the remaining six
subjects, neither ossification nor fibrosis was found, and a
standard scala tympani insertion was performed. Full
insertion of the electrode array was accomplished in all the
cases. Ten of the 12 patients had an uneventful postoperative recovery. Two patients developed a recurrence of
Eur Arch Otorhinolaryngol (2015) 272:3201–3207
3203
Table 1 Demographic data of the 12 Cogan Syndrome patients who received cochlear implants
Patient
Sex
Age at
surgery
(years)
Hearing
loss
Duration of
deafness (months)
1
F
27
Profound
Abnormal
Abnormal
ST ossification
2
F
27
Total
12
Normal
Abnormal
ST ossification
3
4
F
F
41
37
Total
Profound
36
12
Normal
Normal
Normal
Normal
Patent cochlea
Patent
Cochlea
ST
ST
5
M
52
Profound
10
Normal
Normal
ST ossification
ST
N24
72
6
F
44
Profound
28
Normal
Normal
Patent cochlea
ST
Contour
78
7
M
33
Total
24
Normal
Normal
ST ossification
ST
MXM
92
8
F
40
Profound
48
Normal
Normal
Patent cochlea
ST
MXM
94
9
M
23
Total
8
Normal
Normal
ST ossification
ST
MXM
64
10
M
16
Profound
9
Normal
Normal
Patent cochlea
ST
MXM
66
11
F
30
Total
12
Abnormal
Abnormal
ST ossification
ST
MXM
74
12
F
40
Profound
24
Normal
Normal
Patent cochlea
ST
N24
85
6
HRCT
MRI
Intraoperative
findings
Surgical
technique
CI
model
Follow-up
(months)
SV
Contour
158
SV
N24
96
N22
N24
134
124
F female, M male, HRCT high-resolution computed tomography, MRI magnetic resonance imaging, ST scala tympani, SV scala vestibuli
Fig. 1 Pre- and postimplantation individual word
recognition scores
100
90
80
Preop
70
60
1-yr postop
50
40
30
5-yr postop
20
10
0
1
2
3
4
keratitis the day after surgery that was successfully treated
with local atropine and corticosteroid therapy. No patients
in this series experienced flap complications or other local
or systemic complications. Before implantation, the mean
correct score for the 12 patients was 9.7 % (range 0–30 %)
and 10.9 % ?16 (range 0–48 %), on the word and sentence
tests. At 12 months after implantation, the group means for
word and sentence recognition tests were 91.4 % (range
75–100 %) and 93.1 % (range 76–100 %). After 5 years of
implant use, the mean percentage of correct word and
sentence recognition scores was 94 % (range 85–100 %)
and 96.3 % (range 90–100 %). Figures 1 and 2 show
5
6
7
8
9
10
11
12
hearing test scores for the 12 patients before and at 1 and
5 years after implantation.
Discussion
Hearing loss in patients with CS is generally bilateral and
often progresses to irreversible bilateral profound SNHL
despite high-dose corticosteroids and immunosuppressive
treatment [5, 8, 10, 14–18]. Temporal bone histological
analysis in patients with CS has shown (1) atrophy of the
organ of Corti [19, 20], (2) fibrous tissue and bone
123
3204
Fig. 2 Pre- and postimplantation individual
sentence recognition scores
Eur Arch Otorhinolaryngol (2015) 272:3201–3207
100
90
80
70
Preop.
60
50
1-yr postop.
40
5-yr postop
30
20
10
0
1
2
3
4
proliferation that involves the cochlea and vestibular labyrinths [19, 21], (3) demyelinization of the eighth cranial
nerve [20], (4) degeneration of the sensory receptors and
supporting structures of the cochlea and vestibule [19] and,
(5) endolymphatic hydrops [19–21]. These histopathologic
findings suggest that the pathogenesis of SNHL may be
related to an immunologic mechanism secondary to an
inflammatory attack on the membranous labyrinth [7].
Cochlear implantation in CS may be technically difficult
due to the tendency for inflammatory disease to induce
endosteal reaction leading to obliteration of the intracochlear ducts by soft-tissue and bone [21–23]. Since
management of ossified cochlea still represents a surgical
difficulty, early cochlear implantation should be recommended. In our population of 12 patients, 6 presented an
intracochlear obliteration, demanding modifications of the
surgical technique. In four cases, bone obliteration, which
was confined to the first millimeters of the inferior segment
of the basal turn, was successfully managed by tunneling
through the ossified portion of the basal turn. In the other
two patients, the inferior segment of the basal turn was
completely filled by bone tissue, and scala vestibuli
insertion was performed using the technique described by
Steenerson et al. [24, 25]. Pre-operative imaging was normal in 22 of the 24 patients reported in the English literature and abnormal in 2 patients. Intraoperatively, other
four patients were found to have some degree of ossification for a total of six patients with ossification. Five of
these patients had scala tympani electrode insertion by
drilling out the inferior segment of the basal turn and one
patient had scala vestibule insertion. All patients but one
had full electrode insertion.
It has been reported that the basic illness renders
patients affected by CS potentially more susceptible to
complications. Among the potential post-operative
123
5
6
7
8
9
10
11
12
complications, those related to the flap are reported as the
more common [18]. Flap ischemia in a young adult with
CS who had undergone implantation for the second time on
the same side was reported by Kontorinis et al. [5]. Wang
et al. [26] have reported a wound healing disorder and
recurrent episodes of skin infections in one of their four
patients during the post-operative period. The patient
reported by Low et al. [15] experienced a pressure sore of
the occiput from the post-operative head bandage. Skin
atrophy from long-term corticosteroid and immunosuppressant therapy and ischemia caused by vasculitis may be
considered to be adverse factors contributing to the
described flap complications (Table 2).
Diligent planning of a flap with a wide blood supply is
fundamental to avoid ischemia-related complications. In
our 12 patients, we used an inverted ‘‘J’’ shaped flap, which
provided a wide blood supply from the posterior auricular
and occipital arteries. No patient in our series has experienced flap problems. Other potential complications are
related to the worsening of CS symptoms due to the stress
caused by the surgical procedure [17]. Two patients of our
series developed a recurrence of keratitis the day after
surgery. Also, Kontorinis et al. [5] reported of a patient
who suffered from an episode of interstitial keratitis postoperatively which was successfully treated with atropine
and corticosteroids. It is probable that in these patients, an
acute phase of the disease with recurrence of the ocular
inflammation occurred as a result of the stress consequent
to the surgical procedure. To date, very few reports have
specifically focused on the post-implantation outcomes in
patients who become deaf due to CS [4, 5, 8, 10, 14–17].
However, most authors agree to the fact that prognosis of
cochlear implantation with regards to hearing results is
excellent in this population [5, 10, 16, 17]. This can be
explained by the fact that these patients became deaf post-
Age/sex
36/M
38/M
Case 2
Case 3
32/F
Case 4
Case 1
39/M
58/F
30/F
65/F
46/M
58/M
34/F
40/F
Case 2
Case 3
Case 4
Case 5
Case 6
Case 7
35/F
9/F
30F
Case 2
Case 3
Case 4
Bovo et al. [4]
Case 1
48/M
21/F
Case 1
Kontorinis et al. [5]
Kawamura et al. [17]
Case 1
55/F
23/F
Case 1
Wang et al. [16]
Case 1
Vishwakarma et al. [8]
Normal
Normal
Normal
Normal
Normal
Abnormal
Normal
Normal
Normal
Normal
Normal
Normal
Normal
Normal
Normal
Normal
47/F
40/F
Case 2
Case 3
Low et al. [15]
Normal
Normal
56/M
Normal
Normal
Normal
Normal
Pre-operative
imaging
Case 1
Minet et al. [10]
53/F
Case 1
Cinamon et al. [14]
Author
Table 2 Review of the Literature
Nucleus




Nucleus







MED-EL
Nucleus
Nucleus
Nucleus
Nucleus
Nucleus
Nucleus
Nucleus
Nucleus
Device
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
ST
Surgical
technique
Yes
No
Yes
No
No
Yes
No
No
No
No
No
No
No
No
Yes
No
No
No
No
No
No
No
Ossification
90
70
65
95
90
78







90
31*
100
92.5
92.5
42.5

50
40
Open-set word
recognition (%)

99.1
87.7
100
100
79








72*
100
95.5
97


96
86
Open-set sentence
recognition (%)
Tactile and painful stimulation in the
hemiface which required a
disconnection of all the basal electrodes.
Speech perception worsened to closedset word identification
One episode of interstitial keratitis postoperatively. Skin atrophy and infection
above the implant












Pressure sore of the occiput from dressing







Complications
132
12
96
144
192
12








3




24
36
12
Follow-up
(months)
Eur Arch Otorhinolaryngol (2015) 272:3201–3207
3205
123
28
42

* Closed-set auditory alone testing
F female, M male, ST scala tympani insertion, SV scala vestibule insertion, RS right side, LS left side
SV (LS)

Fig. 3 Pre- and post-implantation (at 1 and 5 years) mean word and
sentence recognition scores for the 12 patients


90
Yes
Yes (LS)
ST (RS)
ST (bilateral)
MED-EL
Nucleus
Normal
18/F
Case 3
Abnormal
31/F
Case 2
Complications
Open-set sentence
recognition (%)
Open-set word
recognition (%)
Ossification
Surgical
technique
Device
Pre-operative
imaging
Age/sex
Author
Table 2 continued
123
The patient only reached closed-set word
identification
Eur Arch Otorhinolaryngol (2015) 272:3201–3207
Follow-up
(months)
3206
lingually and are generally implanted after a short period
from the onset of profound SNHL. Minet et al. [10]
described the hearing results obtained in a series of four
patients with CS. At the post-operative evaluation, the
mean open-set word and sentence recognition scores were
about 82 and 97.5 %, respectively. In data on seven
implanted patients with CS provided by Wang et al. [16],
the mean 2-year post-operative open-set sentence recognition score was 98.7 %. However, Bovo et al. [4] reported
a different experience with two of their three CS implanted
patients reaching non-satisfactory outcome. In one patient,
cochlear ossification advanced after implantation with four
electrodes becoming progressively non-functioning and six
electrodes being switched off due to painful sensations.
The remaining patient reached only identification performances: the authors ascribed the limited benefit to an
intervening nerve dyssynchrony or to cognitive/psychological disorders which interfere with auditory processing
[4]. In addition, a post-implant deterioration of speech
perception was observed in one patient with CS by Quaranta et al. [27]. The authors speculated that th…
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