DECEMBER,

THE

ASSOCIATION

OF

CLEIDOCRANIAL HEARING LOSS*

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WITH By

HAROLD

B. HAWKINS, and CHARLES NEW

M.D.,t ROBERT J. PETRILLO, HAVEN,

1975

DYSOSTOSIS

SHAPIRO, M.D4

M.D.,t

CONNECTICUT

ABSTRACT:

Three new cases paper. The significant

of cleidocranial dysostosis points concerning this

is predominantly

malities

a middle

of the

cating either was corrected temporal

(2)

a cochlear surgically

or

which

makes

bone

hearing

loss

cases

with

in the

literature

development

this

i.e.,

to hearing cases, all

with

the are

cephalopelvic

dysostosis only eight

cases

dis-

and

amply of

hearing and den-

considered. cleidocranial

loss has been in the otologic

and

includes

more

and three

three

case

with

tion

in

De

external

hypoplasia.

Reynier7

in 1948

(in

a single

single hearing conduction reported e

From

auditory In

case was

in a slightly hearing

the

fifth

the Departments

case of

the

()

and

the

number

of

of

and

loss.

man by

aid.

1958,

In

in an eleven

row

and

mally

a

whose a bone

long

Otolaryngology3

old

hearing

944

Hospital

a

canals,

was

reported

year external

old girl, auricle,

13

and

a conductive

showed

and

50

hearing deficit respectively.

demonstrated

with bone

external

dense

canal

small

middle

were

clumped

the

ear.

mas-

of

of

the of

Raphael,

New

In

Tema naran

abnor-

addition,

together

handle

process

with

the

in the

attic;

the

malleus

nor

incus

was

identified.

the Haven,

left

temporal

Connecticut.

25

on the Plain

absent pneumatization. laminagraphy revealed

right

of St.

cleidocranial

loss

patient, right

Audiometry

Laminagrams The

improvement. of

ofconductive and left sides,

neither

a few air cells. myringotomy

hearing

external

loss.

normal

conductive showed

with only bilateral

example

with

infec-

with

the tympanic memretracted. Testing by

in a marked seventh

ossicles

Gay3

year

and

external a single

of ear

male

revealed a bilateral Roentgenography

toids poral

mastoid

described

old

audiometry hearing

roentgenograms

hear-

history

year

This the

of

of the reported

however, dull and

decibels right

re-

mixed deafness. pneumatization

no

previous

by F$ns.2 The had a deformed

narrowing

year old improved

Radiologyt

first

family)

had

canals

24

than

ear canals; branes were

dysostosis

new

a mixed

Davis’

1954,

no

narrowed

cleidocranial dysostosis with ing loss. All of these patients of the

loss

of

difficult;

common

a five

resulted

LITERATURE

cases

hearing

sclerosis

and narrowing canals. Jaffee4

The

loss.

PERTINENT

Nager

ear

technically

hearing

ported

middle

is dense

sclerotic mastoids Adenoidectomy

cited in litera-

this association has not been radiologic journals, this paper the roentgen findings in the

hearing

the

there

mastoids auditory

and

associated

dysplasia, disproportion

bone

be

to structural abnorconduction deficit mdi-

girl with moderate patient also had

affects of there

problems

have been the relationship

temporal

is a rare,

In spite findings,

tal dysplasia However,

ture. Because described in will discuss

()

operation

may

membranous

clinical

disease,

()

problem; case;

ear

dysostosis

which

of

proportion, dental loss. Cephalopelvic

problem secondary a small bone

in this deficit

suggests.

bone.5’6’9 roentgenologic few

with

a middle

abnormality

relatively

eighth nerve one reported

dysostosis

inherited cartilaginous striking

an in

cleidocranial

C LEIDOCRANIAL the

ear conduction there is sometimes

ossicles;

with hearing loss are reported association are: (i) the hearing

the bone

VOL.

No.

225,

Cleidocranial

4

Dysostosis

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TABLE

945

I

.

eoe

D.

0

0

Normal

a.

and

Male

Female

CleidoCranial

Dysostosis

CleidoCranial

Dysostosis

I. Five

FIG.

with

additional

members

dysostosis

showed

slight

canal

and

and

ossicles.

narrowing

an

of

abnormally

the

long

ear

process

of

the incus and the handle of the malleus could be identified. The inner ear and porus acousticus were normal bilaterally. The last case, recorded by Pou8 in 1971, Oc-

curred

in a

progressive month she

loss period.

had

old

of On

narrow

Audiometry

deficit

year

23

external showed

on the

left

external

cedure

a

tion,

the

conductive. ear exploration approach.

difficult

of the long

process

ofthe

stapes canal.

of the

stapes

oval

window.

the

malleus

were

The

footplate

and

a wire-vein

ing

loss

Our

mixed

loss

She

on

underthrough

The

to be fixed In addition, was

also

the

stapes

operation, the

right

pro-

all

cranial umented

hearing

of

severe

individuals

At

opera-

incus

was

short,

this Eight

removed

the

conductive improved.

hear-

CASES

D.B.,

(R.S., same

family,

and

audiometrically

wi th

loss.

There

cleidocranial

family over a of the thirteen

four have

and have

documented

dvsostosis

the

introduced.

the

roentgenographically

fixed

and

was

was

patients from

and incus

was

OF

the foot-

thickened.

prosthesis

three

B.C.),

the

considerably

of

on

against the

thickened

Both

REPORT

canal.

because

external

the nerve

plate

cleidocranial

loss.

canals.

decibel

40

causing facial at

documented

hearing

Following

developed

over an eight examination,

an 8o decibel

auditory was

narrowing

who

auditory

and

the right, primarily went a right middle an

girl

hearing physical

have

associated

external middle

LOBS

of family

with

the

small

However,

Hearing

cleidoare

docthirteen

dysostosis generation span. an associated

in

H.

946

B. Hawkins,

R.

Shapiro

and

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to

I1;:.

J.

C.

moderate

mixed

tient

had

Five

additional by

wr,

loss

previous

members

of

history

(Fig.

A

,

FIG.

2.

Towne

projection

showing

marked

of both

petrouS

bones.

R.S.,

D.B.,

and

This

to have

loss.

only

three

who

have

B.C.

been

are

fully

the

passing,

but

patients

mented.

in

sclerosis R.S. history

CASE

2.5 year loss,

more

showed ing

is a 38

I.

gap. Past

marked

in

decibels,

the

with

cephalopelvic

raphy

of

the

thin,

.

thin

bone. of

suture,

the

averagair-bone

Roentgenog-

to

be

being

only

a

left

clavicle,

The

at

infection. sections

clavicles

clavicle

with a pseudoarthrosis. showed many Wormian metopic

ear

decibel

showed

a

Audiometry

left

history ofear three cesarean

right

consisted

with

hearing

ear.

the

a 35-45

chest the

defined,

also

left in

disproportion

hypoplastic,

poorly

woman

progressive

loss

There was no prior history included

for

old

of bilateral

a combined 82

year

least

two

poor

of

the canal to

was

obtain

later,

the

so

narrow

adequate patient

are

hearing

be

limited

of to

the

no

other

our the

Keats’

temporal

a de-

series

the the

mention squamosa

changes. and

in

All

had

petrous

cortical

to

bone because attention in

of

pronounced mastoid

thickening

the

(Fig.

bones The

2).

squamosa the degree

was similarly of squamosal

involved, thickening

although varied

somewhat However,

from patient in all patients,

to patient the thin,

(Fig. 3). normally

radiolucent

squamosa

was

petrosal radiolucent

sclerosis was semicircular

so

stood out in bold relief, newborn skull (Fig. 4).

radiopaque.

The

striking that the canals and cochlea reminiscent

of

the

DISCUSSION

was atcanal.

impossible

have and

segments

pneumatization

months

and

family

associated

and

of

Skull roentgenograms bones, a persistent

the mastoids. A left stapedectomy tempted through the external auditory

However,

due

infection.

FINDINGS

will

Jarvis

“thickening”

docu-

pa-

this

an

of the temporal has received scant

literature. hearing

Neither ear

i).

discussion

scription latter

sclerosis

2975

dysostosis

ROENTGENOLOGIC

&__.__I

DECEMBER,

loss.

of

cleidocranial

known t

hearing

a history

documented

rr

Petrillo

that

dysplasi

a,

cephalopelvic

dispro-

it was

exposure.

underwent

Dental

Two a second

operation. Through a posterior auricular approach, the sclerotic, thickened bony auditory canal was thinned with an electric drill to permit

the

skin

was

entry

of an

then

adequate

elevated

ear

down

speculum.

to the

The

annulus

and

the eardrum reflected forward. This revealed marked thickening of the posterior auditory canal with an extensive overhang, obscuring the

oval

window

and

the

bleeding ensued and expose the oval window Therefore, the operation CASES

R.S.

II

and

III.

it

D.B.

Roentgenography

demonstrated

cleidocranial Audiometry

the

and of

Considerable

the in

both

*#{149}_,.

to

t

B.C. are nieces of chest and skull

characteristic

dysostosis showed

stapes.

was not possible and stapes adequately. was terminated.

of them

findings both

of

patients. to

have

mild

i FIG.

3.

Lateral

view

kj ofskull

demonstrating

increased

density of the temporal squamosa. Normally, is one of the thinnest areas in the calvaria.

this

VoL.

No.

125,

Cleidocranial

4

Dysostosis

947

problem.

The

phy

was

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ossicles

case

in which

showed

clumped

Operative

together

bulky, footplate

window.

It

of the

ossicles

ossicles the stapes

likely

will

cleidocranial

that

4. Coned-down

view

portion,

and

hearing

nificant

clinical

cleidocranial

than

total,

seek out physicians look

in other

dysostosis

against in the audiometry

not

be

disease: rather

inclined

hearing

with

hearing

dysostosis be rare. of the

if

hearing One

which

on

normal

a

few of

a

Hospital Chapel

New

Haven,

eighth

facial

nerve

middle

ear

temporal

bone

a modification

of St. Street

which

ofthe

surgi-

Raphael

o6

Connecticut

DAVIS,

I I

P. L. Deafness

and cleidocranial Arch. Otolaryng., 1954,59,

sis. A.M.A. 2.

FNS,

M.

Ear

at

3.

GAY,

4.

JAFFEE

Acta

S.

Congenital

least is a

LASKER,

tosis.

7.

NAGER, dungen. Suppi.

8.

by

review

9.

of RAD.

Human F. R.,

hororgan is

shoulder-neck auditory 1968, 78, 2I 19-2139. KEATS, T. E. Cleidocranial 40

new

THERAPY

1974, 121, -i6. G. W. Inheritance

MED.,

argues

conduction

J. L., and

JARvIS,

ROENTGENOL.,

6.

nerve.

cleidocranial

1969,

Laryngoscope,

dysostosis:

The

in

oto-laryng.,

9 I5.

5.

temporal

malformations

dysosto.. 602-603.

67, 483-489. I. Case of dysostosis cleidocranialis with mixed deafness. 7. Laryng. & Otol., 1958, 72,

dysostosis.

of

loss,

abnormality that sclerotic the

correcof the

approach.

1350

loss

this. The predominant component hearing loss in all cases tested is

The

I .

indicates

of the

of the hearing is important

Harold B. Hawkins, M.D. Department of Radiology

to

problem.

sclerosis

or cochlear conceivable

encroach a

loss

specifically

REFERENCES

is not

are component

a cochlear

of the

only association

deficit

there

cases

ofsurgical knowledge

is

of the

necessitate

anomalies.

conduction or

(2)

(,)

and

bone to a neural unknown. It is However,

in this is partial

association

a neural

may

three

sclerosis

may

fixation the oval

paucity

the

relationship

bone

are the

with

it;

etiology

either

with

dysostosis for

bone

There explain

may

entirely clear although two factors involved. small

sig-

the

cleidocranial indeed, may The

theonly

medical advice; are aware of

cleidocranial and

are

loss loss

patients

dense cal

associated

may

of hearing the hearing

bone showthe semi-

around

problems

which

reports since

(I)

loss

dysostosis.

possibilities of

left petrous

of

bony thickening (arrow).

attic.

patient

abnormalities

be found

because ofthe possibility tion. Also important

ing the marked circular canals

the

with to

looked for. Appreciation of the nature loss in cleidocranial dysostosis

FIG.

abnormal

in

in another

thick of

seems

laminagra-

dense

visualization

showed of the

of

one

done

J.

cases.

AM.

&

NUCLEAR

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dysos-

Biol., 1946, z8, 103-126. and DE REYNIER, J. P. Das

bei Pract.

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(cleido-cranSurg., 1946,

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