Chronic Pain and Myotonic Dystrophy

A recent article explored Chronic Pain and myotonic dystrophy. (click on link for full study). this is something that many of our patients experience. Heat was being used by 26% of patients. The only treatment that was relatively highly effective and was still being used by a substantial number of patients (26%) was heat. Perhaps this is because heat is an extremely accessible treatment (most people own a hot water bottle or heating pad) that has few, if any, negative side effects.

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Otitis media. – Ear infections

Is currently the most common childhood disease with peak incidence in the first two years of life. Although OM during childhood is nearly universal, about 5-20% of children have recurrent OM and chronic OME. Besides age, several characteristics have been identified as risk factors of OM, including male gender, Caucasian, and Native American race, and family history of OM. Children with CMD are at risk for OM. Why is this? Two theories. One is that the growth of the skull with Children with CMD may affect the drainage of the ear. The other theory is that the muscle that regulates the Eustachian tube does not function properly. The Eustachian tube has 3 principal functions of

 Allowing air into the ear to ventilate
 Protection of the ear  (closing tube off)
 Allowing fluids to drain from the ear

It is still not know why ear infections continue to plague children with CMD.


Defined: Otitis Media (Ear infection) is defined as an inflammation of the middle ear.

Recurrent Otitis Media: There are several definitions.. Three episodes of OM before age 1,3, 0r 7……..Six or more episodes before age 6…… six or more episodes in a 12 month period

Otitis Media  can be observed in children with and without symptoms. The most common symptoms will be related to respiratory infections. Other symptoms may include earache, loss of appetite, fever, restless, sleeplessness

Antibiotic Treatment:

Antibiotic treatment is becoming more complex and more bacteria have a high level resistance or reduced susceptibility to commonly employed antibiotics.  Children may also benefit from antibiotics given prophylacticly (as a precaution against infection returning) Sulfoamides and Amoxicillin would be the two agents of choice according to   Roberts. Amoxicillin should be restricted, however, as it might promote resistant bacteria. Sulfoamides showed an 86% reduction in OM in one study.

Surgery-Placing Ear Tubes

Myringotomy with Tympanostomy tube insertion is the most common surgical procedure performed in children requiring general anesthesia. The reported rate of Myringotomy in 1986 in USA was 58,000 cases. The procedure is basic to cut a small slit in the eardrum and to insert a small tube to allow for ventilation and drainage. The procedure is almost without pain. For older folks who can hold still it can be done without a general anesthetic. For kids a general anesthetic is required in most cases. The tube will stay in for about 6 moths unless a long term tube is inserted,

Indications for Surgery: Recurrent Otitis Media refractory (not responding to) to antibiotic therapy, persistent OEM for greater than 3 months with associated hearing loss greater than 20dB

Tubes can be of Short term or long term insertion. Longer term tubes might be indicated if recurrent Otitis Media continues to occur. There are some downsides to longer term insertion though. Some studies follow on long term tube


Management of Otitis Media for CMD

First parents must be very diligent in reviewing this problem  as the chance for Ear Infections is very high. Sometimes the children will not report the symptoms with OM. My son Christopher for example we find out that he has this condition when he stops eating, is listless, and has a temperature. When we even suspect this we make sure that he is seen that same day and antibiotics started.

Chris has had 5 sets of ear tubes put in all of the short variety. These seem to immediately clear up his ear infections. However, they have only stayed in for a short period of time and then they disappear. You have to be careful about getting water in the ear with swimming and showering. Chris had the more secure T tubes placed when he was 9. These work well and will stay in for a longer period of time


Hints: Find a hospital and doctor that is willing to work with you. You will need to educate them on this subject and particularly that kids with CMD have high susceptibility to this disease. We knew very often that Chris would have an ear infection and would make an immediate that day appointment with the pediatrician.

For Surgery find a kid friendly hospital. We have gone to two hospitals. One required a lot of pre surgical workup including drawing blood. Chris has tiny veins and it was much more traumatic to draw the blood than to have the surgery. Find a center that does not require this for young kids with ear tubes.

Remember that you can do ear tubes in a doctors office and it is a relatively easy procedure. You just can move at all during the procedure. Therefore kids have to be put under anesthesia. But the procedure only takes 5-10 minutes at most.

So insist on some kid friendly procedures or find another center!


Make sure that you are actively managing your children’s medical care and that the doctor knows that CMD has a high correlation with ear infections.

Long-term middle ear ventilation with T tubes: The perforation problem


By Richard L. Goode, MD, Stanford, California

Some 1 million ventilation tube insertions are performed each year in the United States. The majority of these are for otitis media with effusion (OME) in children using tubes that remain in place for an average of 6 to 8 months. Because the failure rate of these short-term tubes is approximately 20%, the otolaryngologist must decide in unsuccessful cases whether to again try medical treatment, reinsert a short-term tube, or switch to a long-term tube, such as a T tube, which will remain in place an average of 36 months. By arbitrary definition, short-term tubes are those that remain in place for less than 12 months, whereas long-term tubes remain longer than 12 months, usually an average of 24 to 36 months.

Although the obvious surgical solution to failure of a short-term tube is to increase ventilation time by inserting a long-term tube, long-term tubes produce certain complications that increase with time; the most serious of these is permanent tympanic membrane (TM) perforation after removal or extrusion of the tube. Although this outcome may be desirable in some cases, it usually requires later closure with a myringoplasty after recovery of eustachian tube function.

There are a number of reports in the literature on the incidence of permanent TM perforation after long-term (years) middle ear ventilation with the T tube. In 12 studies the incidence of this complication varied from 3.0% to 47.5% (3.0% and 3.8% in 2, 5.5% to 7.1% in 6,12.0%to 16.7% in 3, and 47.5% in 1 study), with a mean of 11.5%. If we eliminate the lowest (3%)and the highest (47.5%) studies, the mean is 8.8%. This is significantly higher than the 0.5%to 2.0% perforation incidence reported for short-term tubes (although in some series the incidence is much higher).

Although the reasons for the differences between series are not clear, some principles are evident that can be applied to achieve a low incidence of perforation.

Status of the TM

Insertion of a T tube through a damaged TM, because of either previous tube placement or middle ear disease, appears to produce a higher incidence of perforation. Matt et al. found a 0% perforation incidence when the TM was minimally retracted and a 21% incidence with marked TM retraction. If at all possible, the T tube should be inserted through a normal area of the TM; if the entire TM is abnormal, a higher incidence of early extrusion and perforation should be expected.

Duration of Ventilation

In individual OME cases, the minimum ventilation time needed to achieve a cure is not known. This is particularly true when a previous tube has failed, presumably because it came out too soon. This suggests that a longer ventilation time is required but does not define how long. Because the incidence of perforation increases the longer a tube is in place, another way to decrease this complication is to remove the T tube sooner than the average 36 month retention time. What is a reasonable treatment time that is likely to be successful with an acceptable perforation incidence? In the usual case, I would suggest 18 months as the time to consider removal. This period is about three times longer than the average duration of a short-term tube and would be expected to produce a perforation incidence about three times that of a short-term tube, around 4% to 6%, all else being equal. In my opinion, this is an acceptably low perforation rate. After this time period, the incidence gradually increases to the 8.8% level (average of 10 studies previously noted) at 24 to 36 months and then levels off.

Inglis et al. found that the average time for T tube retention in ears with no perforations was 17.5 months, whereas the average time in the perforation group was 21.5 months. They also recommended consideration of removal at 18 months.

Because the T tube has the unique property that it can usually he removed painlessly in the office without anesthesia, its duration is under the control of the physician. Unfortunately, it may not be obvious whether 18 months is an adequate duration so that tube removal is not followed by another recurrence of OME. In many cases it may be wiser to warn the parents of the increasing complication rate but to leave the tubes in place. Moving the tubes from one TM site to another at 18 months is a consideration but would require another anesthetic.

Other factors are important in this decision, including age, size, other tube complications (drainage, plugging), time of year, and so forth. Regular eustachian tube testing during the ventilation period has been advocated by some authors to determine whether adequate function has returned. Although such testing can be helpful in the removal decision, repeated eustachian tube testing is not routinely used.

Early summer is a good time for removal because OME and upper respiratory infections are less common and water sports are more popular. In bilateral cases one tube can be removed at a time; the second tube is removed 4 to 6 weeks later if the first ear is doing well. If the

OME recurs, the tubes are reinserted at a different site in the TM.

Not All T tubes Are Alike

The “softness” of the tube and the length of the flanges can be factors and may differ from manufacturer to manufacturer. For example, silicone rubber can vary from a durometer of 15 or so (soft) to 45 (hard); softer and shorter flanges appear to produce less damage to the TM on extrusion. Although no definitive data are available, it is my impression that the average retention time correlates well with flange length; shortening the flange length by half, from 4 to 2 mm, decreases retention time by half, all else being equal. This would decrease the T tube average retention time from 36 to 18 months. A tube with 3-mm flanges would remain in place for 27 months, whereas a tube with 1.5-mm flanges would remain in place for about 13 months.

Removal Versus Extrusion

Removal of T tubes appears to produce fewer perforations than extrusion. Matt et al. found a 0% incidence of perforation when T tubes were removed, rather than allowed to extrude. Removing a tube earlier rather then waiting for later extrusion may be more important than increased TM trauma caused by extrusion.

Risk/Cost Considerations

In adults, tubes can be inserted at the physician’s office with the patient under local or topical anesthesia, so cost and risk are relatively low. This is not the case in young children or infants, in whom a general anesthetic and surgical facility are required, increasing the risk and cost. The goal is to produce a cure with a minimal number of procedures. Certainly two or more short-term tube insertions are not as desirable as one long-term tube insertion,assuming the same cure rate. Are two short-term tube insertions much different from one long-term tube insertion and a later fat plug or fascia myringoplasty? Assuming two general anesthetics in each case, the difference to the patient may be small. Cost may vary depending on whether a “package deal” is available for tube insertion but not for myringoplasty.

OME cases suspected of requiring long-term ventilation for cure (children younger than 2years and children with craniofacial anomalies, cleft palates, allergies, etc.) should have along-term tube inserted as the primary tube to minimize the failure rate. As the risk of perforation increases with time, the tubes can be moved, removed, or left in place, depending on the ease.

In summary, I suggest that the best treatment solution after failure of a short-term tube for OME in children is to insert a soft-flanged T tube, ideally though a normal area of the TM, and to reassess it at 6-month intervals regarding need. Shortening the flanges to 2 to 3 mm maybe advantageous in some cases. At around 18 months, assuming no evidence of continuing eustachian tube dysfunction, tube removal in the office can be considered and discussed with the parents. If perforation rates greater than 4% to 6% are considered too high, the tubes can be removed; otherwise they should stay until the physician believes a cure has been obtained. It is hoped that with improved eustachian tube testing, identification of when a tube can be removed will become a science rather than an art, as it is now.


Lowering the Incidence of Perforation Following Goode T-Tube Removal

Presented at the American Society of Pediatric Otolaryngology Annual Meeting May 19th,

1990, Toronto, Canada

by Andrew F. Inglis, Jr., M.D.; Mark A. Richardson, M.D.; Taya S. Higgins, R.N.


A recent report has described an alarmingly high incidence (12%) of tympanic membrane perforation following T-tube insertion. This prompted a prospective study of our experience. All patients at Children’s Hospital and Medical Center undergoing elective removal of T-tubes by the first author between 6/1/89 and 8/31/89 were evaluated for tympanic membrane perforations. In cases where spontaneous extrusion occurred in the contralateral ear, the findings in these ears were included as well. Follow-up was performed at one month in all patients and at three months in patients with tympanic membrane perforations. One hundred four (104) ears completed the study. Seven ears had persistent perforations, for a perforation rate of 6.7%. One of these perforated ears had a mild (22dB PTA) hearing loss. Our current indication for T-tube placement and potential mechanisms leading to the formation of tympanic membrane perforations following T-tube removal will be discussed. We attribute our lowered incidence of perforation to two factors which greatly reduced the trauma to the tympanic membrane following T-tube removal: first, we used a soft silastic tube with short flanges: second, prior to T-tube removal, we pre-treated the ear with a non-ototoxic antibiotic eyedrop for three days to soften the adhesive crust which forms between the tube and the tympanic membrane.

Tympanostomy tube placement is an effective surgical procedure for reducing the incidence of recurrent acute otitis media, and for reversing the hearing loss of otitis media with effusion. Several styles of tympanostomy tubes are available but they generally fall into one of two categories: 1) those which usually extrude spontaneously and 2) those which usually require removal following presumed maturation of the patient’s immune system and eustachian tube function. Soft silicone T-tubes (SSTT) fall in the latter category. They have the advantages of a relatively low spontaneous extrusion rate and removal that can be simply and safely performed in the office. The main disadvantage of these tubes is the previously reported high rate (12%) of residual tympanic membrane™ perforations following removal. This report instigated our own investigation.

Materials & Methods

Fifty-eight consecutive patients undergoing elective removal of SSTT’s between 6/1/89 and 8/31/89 were enrolled in the study. The overwhelming majority of these patients had undergone SSTT placement with short-flanged soft silicone Goode T-tubes (Medco Catalogue Number T5030) which were shortened to about 3 mm. intraoperatively prior SSTT tube insertion. Ears were pre-treated for three days immediately prior to SSTT removal with Sulfacetamidel prednisolone (Blephamide®) ophthalmic drops three times daily and just before entering the exam room. Patients were restrained as necessary and the SSTT’s were visualized with an operating microscope and removed with cup forceps. The integrity of the TM was checked one month following SSTT removal. Patients with intact TM’s had an audiogram at this time, if requested by the family. Patients with residual perforations were asked to continue water precautions and a follow-up examination was performed in another two months. The integrity of the TM was reassessed and audiograms were obtained. Patients were considered to have a residual perforation if the TM had not healed by the second follow-up visit, or if the TM had not healed at the first follow-up visit and they failed their  second appointment. Patients were excluded who failed all follow-up. In two instances, because of great traveling distances involved, follow-up was obtained by telephoning the patients’ private physicians. There were a few instances of spontaneous extrusion of the tube in the contralateral ear. The findings in these ears were also included in the study.


Patients Entering Study:


Patients Completing Study:


Healed TM’s:

N=97 (93.30%)

Residual Perforations:

N=7 (6.7%)

Average PTA of Perforated TM’s:

15 dB

Average PTA of Contralateral, Healed TM’s:

14.5 dB

Average Intubation in Healed TM’s:

17.5 mos. (7-40 mos.)

Average Intubation in Perforated TM’s:

21.5 mos. (10-40 mos.)

Spontaneous Extrusions:

N=10 (9.60/0)




Results are summarized in the table above (Table 1). A total of 58 patients with 115 ears were entered into the study. One hundred and four ears met our criteria for completing the study. There were no instances of significant trauma to the ear canal or TM from patient movement during removal. Ninety seven (93.3%) TM’s were intact at the completion of the study. There were seven ears (6.7%) with residual perforations. Of these, two failed their second follow-up visit. Only one ear with a residual perforation had a PTA of greater than 20 dB. This patient’s PTA was 22. The average PTA in ears with residual perforations was 15 dB, and the average PTA of the contralateral healed TM’s was 14.5 dB. In one patient, hearing in the healed ear was worse because of ongoing Eustachian tube dysfunction. Due to failed appointments, three ears with residual perforations were not tested audiometrically. The average age of the patient at the time of tube insertion in the patients with intact TM’s was 25.0 months and in patients with residual perforations was 24.5 months. The average length of time that the SSTT remained in TM’s which eventually healed was 17.5 months (range 7 months to 40 months) compared with 21.5 months (range 10 months to 40 months) in patients with residual perforations. There were 10 ears in which a spontaneous extrusion occurred. This represented an extrusion rate of 9.6%. None of these ears had residual perforations.


As previously mentioned, SSTT’s have the distinction of being easily removable in an office setting with a low spontaneous extrusion rate. As Dr. Goode has pointed out, this is advantageous both when the anticipated need for adjuvant middle ear ventilation is on a short or long-term basis. This tube most closely approaches the ideal situation where intubation time is determined by the otologist rather than the vagaries of spontaneous extrusion.  For most indications, we find a tube with a 12% rate of residual perforation, as previously= reported, unacceptably high.

However, lower rates of residual perforation can be achieved. We attribute our lower rate of residual perforation to our efforts to reduce the tube trauma to the TM. This includes the use of soft silicone style ear tubes with shortened flanges and the care taken to soften the adhesive crust which forms between the ear tube and the tympanic membrane prior to removal of the tube.

Tube re-insertion rates should be markedly decreased with a longer-acting tube. In general, around 20% of patients will require multiple insertions when using spontaneously-extruding tympanostomy tubes. We believe this is in part because of the relatively short tube retention time of tubes such as the Shepard, Armstrong, or Donaldson variety. Over 40 % of the formern two tube types will be extruded in nine months. When SSTT’s are inserted in the young.ear patient for recurrent acute otitis media, one can generally count on the patient’s middle ears remaining ventilated through two consecutive otitis media (winter) seasons. This is not the case with the spontaneously extruding tubes. The overall incidence for re-operation (either repeat ear tube placement or closure of a residual perforation) is probably reduced in select populations when the longer acting SSTT’s are used.  The residual perforation left following removal of SSTT’s is generally not a severe  a few decibels of hearing loss. The perforation assures continued ventilation of the middle ear space, and in cases of unilateral residual perforation, the perforated ear may be more trouble-free than the opposite ear. Corrective myringoplasty generally requires only a fat patch technique and can occasionally be performed in the office. The main drawback to a residual perforation is the need for continued water precautions until the patient’s eustachian tube function enough to promote a high rate of successful myringoplasty.

The factors causing the higher incidence of tympanic membrane perforation with SSTT’s are unclear. Spontaneously-extruding tympanostomy tubes achieve extrusion and TM integrity simultaneously. With SSTT’s, these events are separate. Another factor is the length of the time that the ear is intubated. The longer intubation time obtained with SSTT’s may encourage maturation or stabilization of the mucocutaneous junction of the perforation preventing healing of the TM following SSTT removal. We now generally limit our intubation time to 18 months. Another cause of residual perforation may be the actual trauma of SSTT removal. There usually is an adherent crust connecting the TM to the SSTT. Before our practice of dissolving or at least softening this crust with eyedrops, large portions of the TM were frequently avulsed during tube removal. This rarely occurs following proper softening of the crust. A further source of trauma may be from the stiffer plastic and longer flanges used in other forms of T-tubes prompting our use of softer short-flanged tubes.

There is an unusual situation which uniformly results in a residual perforation. Following a bout of otitis media, the TM may fail to heal around the shaft of the SSTT and on exam the tympanostomy tube appears to be located within a larger perforation. The etiology is obscure, but may be related to partial necrosis of a vulnerable TM area during the infection.  Given our experience with a lower incidence of residual perforation and the prospect of reducing reintubation, we have the following indications for SSTT usage:

1.Patients with craniofacial abnormalities such as cleft palate in whom long-term middle ear ventilation requirements are anticipated;

2.Patients under one year of age with recurrent acute otitis media (we anticipate the need for adjuvant middle ear ventilation will be at least two winter seasons in these patients);

3.Patients who have a history of worsening of recurrent acute otitis media in the winter months yet require ear tube placement in the late winter, spring, or early summer thus increasing the likelihood the middle ear will remain ventilated through the following winter

4.Patients with attenuated or atelectatic TM’s in whom long-term middle ear ventilation is anticipated.


Surgery-Placing Ear Tubes
Indications for Surgery:
Management of Otitis Media for CMD
Long-term middle ear ventilation with T tubes: The perforation problem
Lowering the Incidence of Perforation Following Goode T-Tube Removal